wikidoc - User contributions [en]

Aortic dissection overview

2019-06-26T23:20:51Z

Devesh Rai: /* Aortography */

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__NOTOC__
{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.<ref name="pmid14715319">{{cite journal| author=von Kodolitsch Y, Nienaber CA, Dieckmann C, Schwartz AG, Hofmann T, Brekenfeld C et al.| title=Chest radiography for the diagnosis of acute aortic syndrome. | journal=Am J Med | year= 2004 | volume= 116 | issue= 2 | pages= 73-7 | pmid=14715319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14715319 }} </ref>

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].<ref name="pmid11418429">{{cite journal| author=LePage MA, Quint LE, Sonnad SS, Deeb GM, Williams DM| title=Aortic dissection: CT features that distinguish true lumen from false lumen. | journal=AJR Am J Roentgenol | year= 2001 | volume= 177 | issue= 1 | pages= 207-11 | pmid=11418429 | doi=10.2214/ajr.177.1.1770207 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11418429 }} </ref>

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.<ref name="pmid23701088">{{cite journal| author=Freeman LA, Young PM, Foley TA, Williamson EE, Bruce CJ, Greason KL| title=CT and MRI assessment of the aortic root and ascending aorta. | journal=AJR Am J Roentgenol | year= 2013 | volume= 200 | issue= 6 | pages= W581-92 | pmid=23701088 | doi=10.2214/AJR.12.9531 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23701088 }} </ref>

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].<ref name="pmid21466934">{{cite journal| author=Kienzl D, Prosch H, Töpker M, Herold C| title=Imaging of non-cardiac, non-traumatic causes of acute chest pain. | journal=Eur J Radiol | year= 2012 | volume= 81 | issue= 12 | pages= 3669-74 | pmid=21466934 | doi=10.1016/j.ejrad.2011.02.042 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21466934 }} </ref>

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.<ref name="pmid8416269">{{cite journal| author=Cigarroa JE, Isselbacher EM, DeSanctis RW, Eagle KA| title=Diagnostic imaging in the evaluation of suspected aortic dissection. Old standards and new directions. | journal=N Engl J Med | year= 1993 | volume= 328 | issue= 1 | pages= 35-43 | pmid=8416269 | doi=10.1056/NEJM199301073280107 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8416269 }} </ref>

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

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Aortic dissection overview

2019-06-26T23:19:46Z

Devesh Rai: /* MRI */

<div style="-webkit-user-select: none;">
{|class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=vrbsxsadiwI|350}}
|-
|}
__NOTOC__
{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.<ref name="pmid14715319">{{cite journal| author=von Kodolitsch Y, Nienaber CA, Dieckmann C, Schwartz AG, Hofmann T, Brekenfeld C et al.| title=Chest radiography for the diagnosis of acute aortic syndrome. | journal=Am J Med | year= 2004 | volume= 116 | issue= 2 | pages= 73-7 | pmid=14715319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14715319 }} </ref>

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].<ref name="pmid11418429">{{cite journal| author=LePage MA, Quint LE, Sonnad SS, Deeb GM, Williams DM| title=Aortic dissection: CT features that distinguish true lumen from false lumen. | journal=AJR Am J Roentgenol | year= 2001 | volume= 177 | issue= 1 | pages= 207-11 | pmid=11418429 | doi=10.2214/ajr.177.1.1770207 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11418429 }} </ref>

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.<ref name="pmid23701088">{{cite journal| author=Freeman LA, Young PM, Foley TA, Williamson EE, Bruce CJ, Greason KL| title=CT and MRI assessment of the aortic root and ascending aorta. | journal=AJR Am J Roentgenol | year= 2013 | volume= 200 | issue= 6 | pages= W581-92 | pmid=23701088 | doi=10.2214/AJR.12.9531 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23701088 }} </ref>

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].<ref name="pmid21466934">{{cite journal| author=Kienzl D, Prosch H, Töpker M, Herold C| title=Imaging of non-cardiac, non-traumatic causes of acute chest pain. | journal=Eur J Radiol | year= 2012 | volume= 81 | issue= 12 | pages= 3669-74 | pmid=21466934 | doi=10.1016/j.ejrad.2011.02.042 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21466934 }} </ref>

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:18:53Z

Devesh Rai: /* CT */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.<ref name="pmid14715319">{{cite journal| author=von Kodolitsch Y, Nienaber CA, Dieckmann C, Schwartz AG, Hofmann T, Brekenfeld C et al.| title=Chest radiography for the diagnosis of acute aortic syndrome. | journal=Am J Med | year= 2004 | volume= 116 | issue= 2 | pages= 73-7 | pmid=14715319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14715319 }} </ref>

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].<ref name="pmid11418429">{{cite journal| author=LePage MA, Quint LE, Sonnad SS, Deeb GM, Williams DM| title=Aortic dissection: CT features that distinguish true lumen from false lumen. | journal=AJR Am J Roentgenol | year= 2001 | volume= 177 | issue= 1 | pages= 207-11 | pmid=11418429 | doi=10.2214/ajr.177.1.1770207 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11418429 }} </ref>

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].<ref name="pmid21466934">{{cite journal| author=Kienzl D, Prosch H, Töpker M, Herold C| title=Imaging of non-cardiac, non-traumatic causes of acute chest pain. | journal=Eur J Radiol | year= 2012 | volume= 81 | issue= 12 | pages= 3669-74 | pmid=21466934 | doi=10.1016/j.ejrad.2011.02.042 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21466934 }} </ref>

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:18:03Z

Devesh Rai: /* Echocardiography */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.<ref name="pmid14715319">{{cite journal| author=von Kodolitsch Y, Nienaber CA, Dieckmann C, Schwartz AG, Hofmann T, Brekenfeld C et al.| title=Chest radiography for the diagnosis of acute aortic syndrome. | journal=Am J Med | year= 2004 | volume= 116 | issue= 2 | pages= 73-7 | pmid=14715319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14715319 }} </ref>

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].<ref name="pmid21466934">{{cite journal| author=Kienzl D, Prosch H, Töpker M, Herold C| title=Imaging of non-cardiac, non-traumatic causes of acute chest pain. | journal=Eur J Radiol | year= 2012 | volume= 81 | issue= 12 | pages= 3669-74 | pmid=21466934 | doi=10.1016/j.ejrad.2011.02.042 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21466934 }} </ref>

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:16:52Z

Devesh Rai: /* Chest X-ray */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.<ref name="pmid14715319">{{cite journal| author=von Kodolitsch Y, Nienaber CA, Dieckmann C, Schwartz AG, Hofmann T, Brekenfeld C et al.| title=Chest radiography for the diagnosis of acute aortic syndrome. | journal=Am J Med | year= 2004 | volume= 116 | issue= 2 | pages= 73-7 | pmid=14715319 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14715319 }} </ref>

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:15:48Z

Devesh Rai: /* Electrocardiogram */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:14:28Z

Devesh Rai: /* Physical Examination */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid11909573">{{cite journal| author=Bossone E, Rampoldi V, Nienaber CA, Trimarchi S, Ballotta A, Cooper JV et al.| title=Usefulness of pulse deficit to predict in-hospital complications and mortality in patients with acute type A aortic dissection. | journal=Am J Cardiol | year= 2002 | volume= 89 | issue= 7 | pages= 851-5 | pmid=11909573 | doi=10.1016/s0002-9149(02)02198-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11909573 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:13:17Z

Devesh Rai: /* Physical Examination */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:12:23Z

Devesh Rai: /* Physical Examination */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref><ref name="pmid26205591">{{cite journal| author=Pape LA, Awais M, Woznicki EM, Suzuki T, Trimarchi S, Evangelista A et al.| title=Presentation, Diagnosis, and Outcomes of Acute Aortic Dissection: 17-Year Trends From the International Registry of Acute Aortic Dissection. | journal=J Am Coll Cardiol | year= 2015 | volume= 66 | issue= 4 | pages= 350-8 | pmid=26205591 | doi=10.1016/j.jacc.2015.05.029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26205591 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
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[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T23:10:20Z

Devesh Rai: /* Surgery */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]].<ref name="pmid15618075">{{cite journal| author=Chiappini B, Schepens M, Tan E, Dell' Amore A, Morshuis W, Dossche K et al.| title=Early and late outcomes of acute type A aortic dissection: analysis of risk factors in 487 consecutive patients. | journal=Eur Heart J | year= 2005 | volume= 26 | issue= 2 | pages= 180-6 | pmid=15618075 | doi=10.1093/eurheartj/ehi024 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15618075 }} </ref> These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
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[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T22:59:45Z

Devesh Rai: /* Medical Therapy */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used.<ref name="pmid20233780">{{cite journal| author=Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE et al.| title=2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. | journal=Circulation | year= 2010 | volume= 121 | issue= 13 | pages= e266-369 | pmid=20233780 | doi=10.1161/CIR.0b013e3181d4739e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20233780 }} </ref>[[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-26T22:59:05Z

Devesh Rai: /* History and Symptoms */

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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain in the chest or abdominal area depending on the area of involvement. The pain typically radiates to the back and occasionally radiates to the groin area in cases when dissection is progressing distally.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] Angiography scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] or [[cardiac tamponade]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[CT angiography|CT Angiography]], [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses. Often times when patient present with coronary occlusion secondary to dissection, EKG changes suggestive of myocardial ischemia,

patient end up getting [[Coronary anguiography|coronary angiography.]]

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair. The repair can be done via open surgery vs endovascular repair depending on the extent of dissection.

Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

Aortic dissection is the only hypertensive emergency where target BP is less than 120/80 within first four hours of presentation. Ideally, target is to control the heart rate to less than 60 per minute. The first line of agents are beta blockers, [[esmolol]] continuous infusion is used preferably as it is short acting and works for blood pressure as well as heart rate. IV [[Labetalol]] or PO [[propranolol]] or [[metoprolol]] can also be used. Addition of other IV continuous [[nitroprusside]] can also be used. [[Cyanide toxicity]] is a concern when nitroprusside is used for more than 24 hour.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-21T15:00:25Z

Devesh Rai: /* Physical Examination */

<div style="-webkit-user-select: none;">
{|class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=vrbsxsadiwI|350}}
|-
|}
__NOTOC__
{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females.<ref name="pmid15197151">{{cite journal| author=Nienaber CA, Fattori R, Mehta RH, Richartz BM, Evangelista A, Petzsch M et al.| title=Gender-related differences in acute aortic dissection. | journal=Circulation | year= 2004 | volume= 109 | issue= 24 | pages= 3014-21 | pmid=15197151 | doi=10.1161/01.CIR.0000130644.78677.2C | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15197151 }} </ref> In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref>

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses.

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair whereas Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-21T14:59:29Z

Devesh Rai: /* Diagnosis */

<div style="-webkit-user-select: none;">
{|class="infobox" style="position: fixed; top: 65%; right: 10px; margin: 0 0 0 0; border: 0; float: right;
|-
| {{#ev:youtube|https://https://www.youtube.com/watch?v=vrbsxsadiwI|350}}
|-
|}
__NOTOC__
{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). Radial-radial delay or radio-femoral delay (deficit) in pulse can be seen in patients depending on the location of dissection. Pulse deficit is commonly seen in females. In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses.

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair whereas Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-21T14:46:12Z

Devesh Rai: /* Screening */

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{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT|CT Angiography]] or [[MRI|MRI Angiography]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses.

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair whereas Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-21T14:43:41Z

Devesh Rai: /* Risk Factors */

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| {{#ev:youtube|https://https://www.youtube.com/watch?v=vrbsxsadiwI|350}}
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__NOTOC__
{{Template:Aortic dissection}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}

==Overview==
[[Aortic]] dissection is a tear in the wall of the [[aorta]] that causes [[blood]] to flow between the layers of the wall of the [[aorta]] and force the layers apart. [[Aortic]] dissection is a [[medical emergency]] and can quickly lead to death, even with optimal treatment. If the dissection tears the [[aorta]] completely open (through all three layers) massive and rapid blood loss occurs. [[Aortic]] dissections resulting in rupture have a 90% [[mortality]] rate even if intervention is timely.

[[Acute]] [[aortic]] dissection is the most common fatal condition that involves the [[aorta]]. The [[mortality]] rate has been estimated to be as high as 1% per hour during the first 48 hours.<ref name="pmid29685932">{{cite journal| author=Evangelista A, Isselbacher EM, Bossone E, Gleason TG, Eusanio MD, Sechtem U et al.| title=Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research. | journal=Circulation | year= 2018 | volume= 137 | issue= 17 | pages= 1846-1860 | pmid=29685932 | doi=10.1161/CIRCULATIONAHA.117.031264 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29685932 }} </ref> Because of the diverse clinical manifestations of [[aortic]] dissection, one needs to maintain a high index of suspicion in patients with not just [[Ddx:Chest Pain|chest pain]], but also those with [[stroke]], [[congestive heart failure]], [[hoarseness]], [[hemoptysis]], [[claudication]], [[superior vena cava (SVC) syndrome]], excruciating abdominal pain or upper airway obstruction. Despite the fact that a noninvasive [[diagnosis]] can be made in up to 90% of cases, the correct antemortem [[diagnosis]] is made less than 50% of the time. Recognition of the condition and vigorous pre-operative management are critical to survival.<ref name="pmid30660330">{{cite journal| author=Huynh N, Thordsen S, Thomas T, Mackey-Bojack SM, Duncanson ER, Nwuado D et al.| title=Clinical and pathologic findings of aortic dissection at autopsy: Review of 336 cases over nearly 6 decades. | journal=Am Heart J | year= 2019 | volume= 209 | issue= | pages= 108-115 | pmid=30660330 | doi=10.1016/j.ahj.2018.11.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30660330 }} </ref>

==Historical Perspective==
DeBakey and Cooley reported the first successful operation for resection and [[graft]] replacement of the [[ascending aorta]] using [[cardiopulmonary bypass]] in 1956.

==Classification==
Several different classification systems have been used to describe [[aortic]] dissections. The systems commonly in use are either based on either the [[anatomy]] of the dissection ([[proximal]], [[distal]]) or the duration of onset of [[symptom]]s ([[acute]], [[chronic]]) prior to presentation.

There are two systems used to classify Aortic Dissection: Stanford and DeBakey classifications.<ref name="pmid12900496">{{cite journal| author=Nienaber CA, Eagle KA| title=Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. | journal=Circulation | year= 2003 | volume= 108 | issue= 5 | pages= 628-35 | pmid=12900496 | doi=10.1161/01.CIR.0000087009.16755.E4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12900496 }} </ref><ref name="pmid16344407">{{cite journal| author=Tsai TT, Nienaber CA, Eagle KA| title=Acute aortic syndromes. | journal=Circulation | year= 2005 | volume= 112 | issue= 24 | pages= 3802-13 | pmid=16344407 | doi=10.1161/CIRCULATIONAHA.105.534198 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16344407 }} </ref><ref name="pmid14261867">{{cite journal| author=DEBAKEY ME, HENLY WS, COOLEY DA, MORRIS GC, CRAWFORD ES, BEALL AC| title=SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. | journal=J Thorac Cardiovasc Surg | year= 1965 | volume= 49 | issue= | pages= 130-49 | pmid=14261867 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14261867 }} </ref>

Stanford classification (classification on the basis of location of dissection): Type A involves ascending aorta, Type B involves descending aorta

DeBakey Classification (classification on the basis of origin of dissection): Type 1 origin from ascending aorta and goes till aortic arch, Type 2 originated and ends in ascending aorta, Type 3 origin in descending aorta and travels distally.

==Pathophysiology==
[[Aortic]] dissection begins as a tear in the [[aortic]] wall in > 95% of patients. The tear is usually transverse, extends through the [[intima]] and halfway through the [[tunica media|media]] and involves ~50% of the [[aortic]] circumference. A inherent weakness in the tunica media layer predisposes patients to develop tear in the intima layer. Two thirds of dissections originate in the [[ascending aorta]], and 20% are in the [[proximal]] [[descending aorta]].<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

==Causes==
Age related changes due to [[atherosclerosis]] and uncontrolled [[hypertension]] are associated with spontaneous dissection, while blunt trauma injury and sudden deceleration in a motor vehicle accident is a major cause of [[aortic]] dissection.

==Differentiating Aortic Dissection from other Diseases==
[[Aortic]] dissection is a life threatening entity that must be distinguished from other life threatening entities such as [[cardiac tamponade]], [[cardiogenic shock]], [[myocardial infarction]], and [[pulmonary embolism]]. An [[aortic aneurysm]] is not synonymous with [[aortic]] dissection. [[Aneurysm]]s are defined as a localized permanent [[dilation]] of the [[aorta]] to a diameter > 50% of normal. Aortic Intramural Hematoma can also progress to dissection if blood pressure is not well controlled,

==Epidemiology and Demographics==
There are approximately 2,000 cases of [[aortic]] dissection in the US per year, and [[aortic]] dissection accounts for 3-4% of [[sudden deaths]]. The peak [[incidence]] is in the sixth and seventh decades, and males predominate 2:1.

== Risk Factors ==
[[Aging]], [[atherosclerosis]], [[diabetes]], [[hypertension]]<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref><ref name="pmid8350637">{{cite journal| author=Spittell PC, Spittell JA, Joyce JW, Tajik AJ, Edwards WD, Schaff HV et al.| title=Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). | journal=Mayo Clin Proc | year= 1993 | volume= 68 | issue= 7 | pages= 642-51 | pmid=8350637 | doi=10.1016/s0025-6196(12)60599-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8350637 }} </ref> and [[trauma]] are common risk factors for aortic dissection. Uncommon risk factors include aortic surgery, [[bicuspid aortic valve]], [[cocaine]], [[coarctation of the aorta]], [[cystic medial necrosis]], [[Ehlers-Danlos syndrome]], [[giant cell arteritis]], [[heart surgery]], [[Marfan’s syndrome]], [[Pseudoxanthoma elasticum]], [[Turner's syndrome]], [[tertiary syphilis]] and the [[third trimester of pregnancy]].<ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid7778824">{{cite journal| author=Elkayam U, Ostrzega E, Shotan A, Mehra A| title=Cardiovascular problems in pregnant women with the Marfan syndrome. | journal=Ann Intern Med | year= 1995 | volume= 123 | issue= 2 | pages= 117-22 | pmid=7778824 | doi=10.7326/0003-4819-123-2-199507150-00007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7778824 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid1993792">{{cite journal| author=Roberts CS, Roberts WC| title=Dissection of the aorta associated with congenital malformation of the aortic valve. | journal=J Am Coll Cardiol | year= 1991 | volume= 17 | issue= 3 | pages= 712-6 | pmid=1993792 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993792 }} </ref><ref name="pmid14975480">{{cite journal| author=Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J et al.| title=Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). | journal=J Am Coll Cardiol | year= 2004 | volume= 43 | issue= 4 | pages= 665-9 | pmid=14975480 | doi=10.1016/j.jacc.2003.08.054 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14975480 }} </ref><ref name="pmid23032325">{{cite journal| author=Carlson M, Airhart N, Lopez L, Silberbach M| title=Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the international turner syndrome aortic dissection registry. | journal=Circulation | year= 2012 | volume= 126 | issue= 18 | pages= 2220-6 | pmid=23032325 | doi=10.1161/CIRCULATIONAHA.111.088633 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23032325 }} </ref>

==Screening==
Screening guidelines state that an [[EKG]] should be obtained for all patients who present with symptoms suspicious for aortic dissection. A chest x ray should be obtained for patients determined to be in low-risk, and moderate-risk categories, and for patients in high-risk categories, [[TEE]], [[CT]] or [[MRI]] should be obtained.

==Natural History, Complications and Prognosis==
[[Aortic]] dissection carries a very poor [[prognosis]]. 90% of patients who are untreated will be dead at one year. Type A dissection is associated with a worse [[prognosis]] than type B dissection. [[Aortic]] dissection can be complicated by extension to the [[coronary artery|coronary arteries]] resulting in [[myocardial infarction]], involvement of the [[aortic arch]] to cause [[stroke]], dilation of the route to cause [[aortic insufficiency]], extension into the [[pericardium]] to cause [[pericardial tamponade]], and [[heart failure]], and [[aortic rupture]].

==Diagnosis==
===History and Symptoms===
67% of patients with [[aortic]] dissection present with [[acute]] [[symptom]]s (<2 weeks), and 33% with [[chronic]] [[symptom]]s (>= 2 weeks). 74% of patients who survive the initial tear typically present with the sudden onset of severe tearing pain.

===Physical Examination===
[[Aortic dissection]] is commonly associated with varying blood pressure (pseudohypotension or [[hypertension]] or [[hypotension]]), [[wide pulse pressure]] (if the [[aortic root]] is involved causing [[aortic insufficiency]]), [[tachycardia]], [[pulsus paradoxus]], [[swollen face]] due to [[superior vena cava]] compression ([[superior vena cava syndrome]]). In proximal dissections involving [[aortic root]], [[aortic insufficiency]] is a [[complication]], and on physical examination an early [[diastolic]] decrescendo [[murmur]], which is best heard in the right second [[intercostal space]] is noted.

===Laboratory Findings===
Routine blood work is usually not helpful and should not delay definitive [[diagnosis|diagnostic studies]] such as a [[CT]] scan and treatment. [[Hemolysis]] can be present as a result of blood in the [[false lumen]]. The presence of an elevated [[CK MB]] may indicate the presence of concomitant [[acute myocardial infarction]] (often a [[right coronary artery]] occlusion due to occlusion of the [[ostium]] of the [[RCA]] by the dissection). [[Hematuria]] may be present and may indicate the presence of [[renal infarction]].

===Electrocardiogram===
[[ST elevation myocardial infarction]] ([[MI]]) due to occlusion by the dissection of the [[coronary artery]] at its [[ostium]] may be present. The [[right coronary artery]] tends to be involved more frequently than the [[left coronary artery]]. [[Electrical alternans]] may be present in the setting of a [[pericardial effusion]] should the dissection have extended into the [[pericardium]].

===Imaging in Acute Aortic Dissection===
There are a wide variety of imaging studies that can be used to [[diagnose]] [[aortic]] dissection, but in general, [[Transesophageal echocardiography (TEE)|transesophageal imaging]] is the imaging modality of choice in the [[acute]]ly ill patient and [[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Chest X-ray===
An increased [[aortic]] diameter is the most common finding on chest [[X ray]], and is observed in up to 84% of patients. A [[widened mediastinum]] is the next most common finding, and is observed in 15-20% of patients. The chest X-Ray is normal in 17% of patients. A [[pleural effusion]] ([[hemothorax]]) in the absence of [[congestive heart failure]] can be another sign of [[aortic]] dissection.

===CT===
A [[CT scan]] can be used to [[diagnosis|diagnose]] [[aortic]] dissection if neither a [[TEE]] nor [[MRI]] is available in a timely fashion, or if there is a contraindication to their performance. An example would be after hours in an emergency room setting. If the results of the [[CT]] scan are non-[[diagnostic]], then [[TEE]] or [[MRI]] should be performed to confirm the [[diagnosis]].

===MRI===
[[MRI]] is the imaging modality of choice in the assessment of longstanding [[aortic]] disease in a patient who has [[chronic]] [[chest pain]] who is [[hemodynamic]]ally stable or for the evaluation of a [[chronic]] dissection.

===Echocardiography===
In the management of the [[acute]] patient with suspected [[aortic]] dissection, a [[TEE|transesophageal echo]] performed [[acute]]ly in the emergency room is the preferred approach. If the patient is [[hemodynamic]]ally unstable, then a [[TEE|transesophageal echo]] can be performed in the operating room as the patient after the patient has been induced and is being prepared for [[surgery]].

===Aortography===
[[Aortography]] is rarely used in the modern era. It can be used of the other imaging modalities are not available or are inconclusive.

===Coronary Angiography===
Pre-operative [[angiography]] has not been associated with improved outcomes in [[retrospective]] analyses.

==Treatment==
===Medical Therapy===
Type A dissections of the [[proximal]] [[aorta]] are generally managed with operative repair whereas Type B dissections of the [[descending aorta]] are generally managed medically. Even patients who are undergoing operative repair require optimal medical management. The two goals in the medical management of [[aortic]] dissection are to reduce [[blood pressure]] and to reduce the oscillatory shear on the wall of the aorta (the shear-force dP/dt or force of ejection of blood from the [[left ventricle]]). The target [[blood pressure]] should be a [[mean arterial pressure|mean arterial pressure (MAP)]] of 60 to 75 mmHg.

===Surgery===
Any dissection that involves the [[ascending aorta]] is considered a [[surgery|surgical]] emergency, and urgent [[surgery|surgical]] consultation is recommended. There is a 90% 3-month [[mortality]] among patients with a [[proximal]] [[aortic]] dissection who do not undergo [[surgery]]. These patients can rapidly develop [[acute]] [[aortic insufficiency]] ([[AI]]), [[tamponade]] or [[myocardial infarction]] ([[MI]]).

===Secondary Prevention===
Proper treatment and control of hardening of the [[artery|arteries]] ([[atherosclerosis]]) and high [[blood pressure]] may reduce risk of [[aortic]] dissection. It is very important for patients at risk for dissection to tightly control their [[blood pressure]]. Taking safety precautions to prevent injuries can help prevent dissections. Many cases of aortic dissection cannot be prevented. If diagnosed with [[Marfan syndrome|Marfan]] or [[Ehlers-Danlos syndrome]], regular follow-up is advisable.

== References ==
{{Reflist|2}}

{{WH}}
{{WS}}
[[CME Category::Cardiology]]

[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Emergency medicine]]
[[Category:Intensive care medicine]]
[[Category:Up-To-Date]]
[[Category:Up-To-Date cardiology]]

Aortic dissection overview

2019-06-21T14:36:34Z

Devesh Rai: /* Risk Factors */

Aortic dissection overview

2019-06-21T14:29:12Z

Devesh Rai: /* Risk Factors */

Aortic dissection overview

2019-06-21T14:25:58Z

Devesh Rai: /* Causes */

Aortic dissection overview

2019-06-21T14:23:29Z

Devesh Rai: /* Pathophysiology */

Aortic dissection overview

2019-06-21T14:22:48Z

Devesh Rai: /* Pathophysiology */

Aortic dissection overview

2019-06-21T14:19:10Z

Devesh Rai: /* Classification */

Aortic dissection overview

2019-06-21T14:17:38Z

Devesh Rai: /* Classification */

Aortic dissection overview

2019-06-21T14:06:14Z

Devesh Rai: /* Overview */

Aortic dissection overview

2019-06-21T14:03:20Z

Devesh Rai: /* Overview */

Aortic intramural hematoma

2019-04-10T05:35:54Z

Devesh Rai: /* Treatment */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

*MR Angiography is the imaging modality of choice for IMH. Howerver, as it requires 25-35 minutes; thus in emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
*When CT or echocardiorgapgy is equivocal, MR should be the next step.
*On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH.
*CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentric aortic wall thickening can be seen as well.
*MR is superior to CT to CT and echocardiography for diagnosis of IMH.

== Treatment ==
=== Medical Therapy ===
*The mainstay of therapy for IMH is control of hypertension as the hypertensive emergency. In patients with IMH, the target blood pressure should be lower than 130/80 mm of Hg. Labetalol IV or infusion can be a treatment of choice as it controls blood pressure as well as heart rate. Drugs causing reflex tachycardia should be avoided.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref><ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref>
*Heart rate should be controlled prior to initiating blood pressure medications.
*Anti-hypertensive medication acts by decreasing the stress in the media thereby preventing the extension of IMH.
*Response to anti-hypertensive can be monitored with a change in blood pressure and heart rate every 30 minutes.
*When patients are clinically stable, they can be closely monitored without proceeding to surgery.

=== Surgery ===
*Surgery is preferred options in patients who are at high risk to proceed to aortic dissection.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref>
*Surgical option depends on the location of IMH. Replacement of aortic root is advised for type A IMH whereas minimal invasive surgery can be performed.<ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref>

=== Prevention ===
*There are no primary preventive measures available for IMH.

*Once diagnosed and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by imaging.

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T05:34:03Z

Devesh Rai: /* Treatment */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

*MR Angiography is the imaging modality of choice for IMH. Howerver, as it requires 25-35 minutes; thus in emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
*When CT or echocardiorgapgy is equivocal, MR should be the next step.
*On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH.
*CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentric aortic wall thickening can be seen as well.
*MR is superior to CT to CT and echocardiography for diagnosis of IMH.

== Treatment ==
=== Medical Therapy ===
*The mainstay of therapy for IMH is control of hypertension as the hypertensive emergency. In patients with IMH, the target blood pressure should be lower than 130/80 mm of Hg. Labetalol IV or infusion can be a treatment of choice as it controls blood pressure as well as heart rate. Drugs causing reflex tachycardia should be avoided.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref>
*Heart rate should be controlled prior to initiating blood pressure medications.
*Anti-hypertensive medication acts by decreasing the stress in the media thereby preventing the extension of IMH.
*Response to anti-hypertensive can be monitored with a change in blood pressure and heart rate every 30 minutes.
*When patients are clinically stable, they can be closely monitored without proceeding to surgery.

=== Surgery ===
*Surgery is preferred options in patients who are at high risk to proceed to aortic dissection.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref>
*Surgical option depends on the location of IMH. Replacement of aortic root is advised for type A IMH whereas minimal invasive surgery can be performed.

=== Prevention ===
*There are no primary preventive measures available for IMH.

*Once diagnosed and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by imaging.

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T05:32:35Z

Devesh Rai: /* Treatment */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

*MR Angiography is the imaging modality of choice for IMH. Howerver, as it requires 25-35 minutes; thus in emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
*When CT or echocardiorgapgy is equivocal, MR should be the next step.
*On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH.
*CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentric aortic wall thickening can be seen as well.
*MR is superior to CT to CT and echocardiography for diagnosis of IMH.

== Treatment ==
=== Medical Therapy ===
*The mainstay of therapy for IMH is control of hypertension as hypertensive emergency. In patients with IMH, the target blood pressure should be lower than 130/80 mm of Hg. Labetatolol IV or infusion can be a treatment of choice as it controls blood pressure as well as heart rate. Drugs causing reflex tachycardia should be avoided.
*Heart rate should be controlled prior to initiating blood pressure medications.
*Anti-hypertensive medication acts by decreasing the stress in the media thereby preventing the extension of IMH.
*Response to anti-hypertensive can be monitored with change in blood pressure and heart rate every 30 minutes.
*When patients are clinically stable, they can be closely monitored without proceeding to surgery.

=== Surgery ===
*Surgery is preferred options in patients who are at high risk to proceed to aortic dissection.
*Surgical option depends on the location of IMH. Replacement of aortic root is advised for type A IMH whereas minimal invasive surgery can be performed.

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Once diagnosed and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by imaging.

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T05:00:56Z

Devesh Rai: /* Imaging Findings */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

*MR Angiography is the imaging modality of choice for IMH. Howerver, as it requires 25-35 minutes; thus in emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
*When CT or echocardiorgapgy is equivocal, MR should be the next step.
*On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH.
*CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentric aortic wall thickening can be seen as well.
*MR is superior to CT to CT and echocardiography for diagnosis of IMH.

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T04:59:36Z

Devesh Rai: /* Imaging Findings */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used

*MR Angiography is the imaging modality of choice for IMH. Howerver, as it requires 25-35 minutes; thus in emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
*When CT or echocardiorgapgy is equivocal, MR should be the next step.
*On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH.
*CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentric aortic wall thickening can be seen as well.
*MR is superior to CT to CT and echocardiography for diagnosis of IMH.
== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T03:32:12Z

Devesh Rai: /* Imaging Findings */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR Angiography are the modality of imaging which can be used

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T02:25:21Z

Devesh Rai: /* Physical Examination */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T02:24:22Z

Devesh Rai: /* Symptoms */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-10T02:23:21Z

Devesh Rai: /* Physical Examination */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with IMH usually appear distressed and it depends on the stage of their presentation.
*Physical examination may be remarkable for:
:*Sweating
:*Tachycardia
:*Hypertension
:*Aortic Regurgitation murmur
:*Radial-radial pulse deficit or Radial-femoral pulse deficit depending on the location of the IMH

=== Laboratory Findings ===
*There are no specific laboratory findings associated with IMH.

*Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T08:45:06Z

Devesh Rai: /* Symptoms */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*Symptoms of IMH may include the following:
:*Sudden onset chest pain
:*Back pain
:*Radiation of pain from chest to back
:*Abdomen pain
:*Sweating

=== Physical Examination ===
*Patients with usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T08:38:09Z

Devesh Rai: /* Diagnostic Criteria */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref>

=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T08:37:44Z

Devesh Rai: /* Diagnosis */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.
=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T08:26:39Z

Devesh Rai: /* Natural History, Complications and Prognosis */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref>
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref>
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref>
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]

=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T08:11:19Z

Devesh Rai: /* Natural History, Complications and Prognosis */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*IMH resolve spontaneously in about 10% of patients.
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.
*Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.
*Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical)

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]

=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T07:59:05Z

Devesh Rai: /* Risk Factors */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref>

== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]

=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T07:55:04Z

Devesh Rai: /* Risk Factors */

{{SI}}
{{CMG}}
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}

==Overview==

*Aortic intramural hematoma is classically abbreviated as IMH.
*It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer.

*Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury.

*Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall.

*The hematoma propagates along the media layer of the aorta.

*Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating [[aortic dissection]].

*Unlike aortic dissection, no intimal flap is present.

*If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]].

*Conservative management is indicated for aortic intramural hematomas of the descending aorta.
==Historical Perspective==
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was Trans-esophageal echocardiography.<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref>

==Classification==
*IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref>
:*Type A IMH: involves the ascending aorta, and may or may not involve descending aorta
:*Type B IMH: involves the descending aorta only distal to left subclavian artery

==Pathophysiology==
*The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tea in the intimal layer.
*Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>
*On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.

==Clinical Features==
Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.

Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref>

Pain is reportedly severe on onset in almost all the cases, abrupt in onset. Radiation of pain is seen 45.9% of patients.

Upto 32% of these patients can present with hypertension, whereas a minority(11.9%) present with hypotension.

Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.

==Differentiating Aortic Intramural Hematoma from other Diseases==
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdomen pain with or without radiation such as:
:*Aortic Dissection
:*Acute Coronary Syndrome
:*Pulmonary Embolism
:*Renal Infarct
:*Mesenetric ischemia
:*Trauma to the back

==Epidemiology and Demographics==
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref>

===Age===
*IMH is more commonly observed among patients age in the range of 60-80 years old.
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Gender===
*IMH is more common in men with 50-81% of cases occuring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

===Race===
*There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref>

==Risk Factors==
*Common risk factors in the development of IMH are hypertension, connective tissue disorder (Marfan's syndrome, Loeys-Sietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.

== Natural History, Complications and Prognosis==
*The majority of patients with [disease name] remain asymptomatic for [duration/years].
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].

== Diagnosis ==
===Diagnostic Criteria===
*The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]

=== Symptoms ===
*[Disease name] is usually asymptomatic.
*Symptoms of [disease name] may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]

=== Physical Examination ===
*Patients with [disease name] usually appear [general appearance].
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]

=== Laboratory Findings ===
*There are no specific laboratory findings associated with [disease name].

*A [positive/negative] [test name] is diagnostic of [disease name].
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
*Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

===Imaging Findings===
*There are no [imaging study] findings associated with [disease name].

*[Imaging study 1] is the imaging modality of choice for [disease name].
*On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

=== Other Diagnostic Studies ===
*[Disease name] may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.

*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action 1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].

=== Prevention ===
*There are no primary preventive measures available for [disease name].

*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

==Examples==

[http://www.radswiki.net Images courtesy of RadsWiki]

[[Image:Intramural-hematoma-006.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-005.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-007.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-002.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-003.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 
[[Image:Intramural-hematoma-004.jpg|thumb|350px|left|Contrast enhanced CT: Aortic intramural hematoma]]
 

* [http://www.ajronline.org/cgi/content/short/181/2/309 Macura, Katarzyna J., Corl, Frank M., Fishman, Elliot K., Bluemke, David A. Pathogenesis in Acute Aortic Syndromes: Aortic Dissection, Intramural Hematoma, and Penetrating Atherosclerotic Aortic Ulcer. Am. J. Roentgenol. 2003 181: 309-316.]
* [http://www.emedicine.com/radio/topic43.htm Gomez-Jorge, Jackeline. E-medicine rads article]

==See also==
*[[Aortic dissection]]
*[[Penetrating atherosclerotic aortic ulcer]]

==External Links==
*[http://www.emedicine.com/radio/topic43.htm E-medicine: Radiology article]
*[http://goldminer.arrs.org/search.php?query=intramural%20hematoma Goldminer: Intramural hematoma]

{{Circulatory system pathology}}
==References==
{{Reflist|2}}

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[[CME Category::Cardiology]]

[[Category:Cardiology]]

Aortic intramural hematoma

2019-04-09T07:15:45Z

Devesh Rai: /* Epidemiology and Demographics */

Aortic intramural hematoma

2019-04-09T07:15:03Z

Devesh Rai: /* Age */

Aortic intramural hematoma

2019-04-09T07:12:51Z

Devesh Rai: /* Age */

Aortic intramural hematoma

2019-04-09T07:03:00Z

Devesh Rai: /* Epidemiology and Demographics */

Aortic intramural hematoma

2019-04-09T07:02:28Z

Devesh Rai: /* Epidemiology and Demographics */

Aortic intramural hematoma

2019-04-09T05:01:40Z

Devesh Rai: /* Differentiating [disease name] from other Diseases */

Aortic intramural hematoma

2019-04-09T04:56:37Z

Devesh Rai: /* Clinical Features */

Aortic intramural hematoma

2019-04-09T04:56:09Z

Devesh Rai: /* Clinical Features */

Aortic intramural hematoma

2019-04-09T04:45:20Z

Devesh Rai: /* Pathophysiology */