Aortic intramural hematoma

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Aortic intramural hematoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and Keywords: : IMH

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 with the medial 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

Classification

IMH is classified into two types on the basis of Standford classification.[3]

Pathophysiology

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%), and abdominal pain (13.1%).[4]
  • Pain is reportedly severe on onset in almost all the cases, abrupt in onset.
  • Radiation of pain is seen 45.9% of patients commonly radiating to the back and shoulders.
  • Up to 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

Epidemiology and Demographics

  • The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.[5]
  • IMH is more commonly observed among patients age in the range of 60-80 years old.
  • Mean age for presentation is 69 years.[6]
  • IMH is more common in men with 50-81% of cases occurring commonly in men.[6]
  • There is no racial predilection for IMH.[6]

Risk Factors

Natural History, Complications and Prognosis

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.[11]

History and Symptoms

Physical Examination

  • Patients with IMH usually appear distressed and it depends on the stage of their presentation.[11]
  • Physical examination may be remarkable for sweating, tachycardia, hypertension, aortic regurgitation murmur, and radial-radial pulse deficit or radial-femoral pulse deficit depending on the location of the IMH.

Laboratory Findings

contrast-enhanced CT[12]
contrast-enhanced CT[13]

Imaging Findings

  • MR angiography is the imaging modality of choice for IMH. However, as it requires 25-35 minutes; thus in an emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
  • When CT or echocardiorgraphy 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 the diagnosis of IMH.
  • CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentic aortic wall thickening can be seen as well.
  • MR is superior to CT to CT and echocardiography for diagnosis of IMH.

Treatment

Medical Therapy

Surgery

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.

Guideline

2014 ESC Guidelines on the Diagnosis and Treatment of Aortic Diseases (DO NOT EDIT)[16]

Recommendations Class Level
  • "In all patients with IMH, medical therapy including pain relief and blood pressure control is recommended."
I C
I C
  • "In cases of Type B IMH, initial medical therapy under careful surveillance is recommended."
I C
I C
  • "In complicatedc Type B IMH, TEVAR should be considered."
IIa C
  • "In complicatedc Type B IMH, surgery may be considered."
IIb C

Abbreviations: CT = computed tomography; IMH = intramural haematoma; MRI = magnetic resonance imaging; TEVAR = thoracic endovascular aortic repair.

See also

External Links

References

  1. Case courtesy of Dr Vincent Tatco, Radiopaedia.org, rID: 48454
  2. Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA; et al. (1997). "Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection". Am Heart J. 134 (3): 495–507. PMID 9327708.
  3. Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA; et al. (2014). "Aortic arch dissection: a controversy of classification". Radiology. 271 (3): 848–55. doi:10.1148/radiol.14131457. PMID 24617732.
  4. 4.0 4.1 4.2 Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V (2014). "Aortic intramural hematoma and its complications". Circulation. 129 (6): 711–6. doi:10.1161/CIRCULATIONAHA.113.001809. PMID 24515957.
  5. Larson EW, Edwards WD (1984). "Risk factors for aortic dissection: a necropsy study of 161 cases". Am J Cardiol. 53 (6): 849–55. PMID 6702637.
  6. 6.0 6.1 6.2 Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA (2016). "Acute Aortic Dissection and Intramural Hematoma: A Systematic Review". JAMA. 316 (7): 754–63. doi:10.1001/jama.2016.10026. PMID 27533160.
  7. Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL; et al. (2000). "The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease". JAMA. 283 (7): 897–903. PMID 10685714.
  8. Braverman AC (2010). "Acute aortic dissection: clinician update". Circulation. 122 (2): 184–8. doi:10.1161/CIRCULATIONAHA.110.958975. PMID 20625143.
  9. Nienaber CA, Sievers HH (2002). "Intramural hematoma in acute aortic syndrome: more than one variant of dissection?". Circulation. 106 (3): 284–5. PMID 12119238.
  10. Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H; et al. (2002). "Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis". Circulation. 106 (3): 342–8. PMID 12119251.
  11. 11.0 11.1 11.2 11.3 Song JK (2004). "Diagnosis of aortic intramural haematoma". Heart. 90 (4): 368–71. PMC 1768152. PMID 15020502.
  12. Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557
  13. Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557
  14. 14.0 14.1 Dake MD (2004). "Aortic intramural haematoma: current therapeutic strategy". Heart. 90 (4): 375–8. PMC 1768168. PMID 15020506.
  15. 15.0 15.1 Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR (2003). "Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery". Tex Heart Inst J. 30 (4): 325–7. PMC 307723. PMID 14677748.
  16. Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwöger M, Haverich A, Iung B, Manolis AJ, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA, Allmen RS, Vrints CJ (November 2014). "2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)". Eur. Heart J. 35 (41): 2873–926. doi:10.1093/eurheartj/ehu281. PMID 25173340.


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