Aortic aneurysm

Resident Survival Guide

Aortic aneurysm
Atherosclerotic Aneurysm: Gross, an excellent example, natural color, external view of typical thoracic aortic aneurysms Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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For patient information on Thoracic aortic aneurysm, click here

For patient information on Abdominal aortic aneurysm, click here

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

Overview

An aortic aneurysm is a general term for any swelling (dilatation or aneurysm) of the aorta, usually representing an underlying weakness in the wall of the aorta at that location. While the stretched vessel may occasionally cause discomfort, a greater concern is the risk of rupture which causes severe pain, massive internal hemorrhage and, without prompt treatment, results in a quick death. Aneurysms often are a source of blood clots (emboli) stemming from the most common etiology of atherosclerosis.

Classification

There are 2 types of aortic aneurysms:

• Thoracic aortic aneurysm (TA), which occur in the thoracic aorta (runs through the chest)
• Abdominal aortic aneurysm (AAA), which occur in the abdominal aorta, are the most common.

Historical Perspective

• Aortic aneurysm was first discovered by Antyllus, a Greek surgeon, in the second century AD.
• In 1555, Vesalius first diagnosed AAA.
• In 1817, the first ligation was developed by Astley Cooper to treat ruptured iliac aneurysm.
• In 1888, Rudoff Matas came up with the concept of endoaneurysmorrhaphy.
  • Livesay JJ, Messner GN, Vaughn WK. Milestones in the treatment of aortic aneurysm: Denton A. Cooley, MD, and the Texas Heart Institute. Tex Heart Inst J. 2005;32(2):130-4.

Classification

• Thoraco-abdominal aorta may be classified according to Crawford classification into 5 subtypes/groups:

• Type 1: from the origin of left subclavian artery in descending thoracic aorta to the supra-renal abdominal aorta.
• Type 2: from the left subclavian to the aorto-iliac bifurcation.
• Type 3: from distal thoracic aorta to the aorto-iliac bifurcation
• Type 4: limited to abdominal aorta below the diaphragm
• Type 5 (Added by Safi's group): from distal thoracic aorta to celiac and superior mesenteric origins, but not the renal arteries.
• http://www.annalscts.com/article/view/1070/1308

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of aortic aneurysm is characterized by progressive dilation, rupture, and dissection.
• The fibrillin-1 gene/Mutation has been associated with the development of thoracic aortic aneurysms in Marfan syndrome
• Other gene mutations coding for collagen, elastin and other elements of the extra-cellular matrix have been associated with the development of aneurysms in some genetic disorders, including Ehlers-Danlos and Alport syndromes among others.
• Mutations in the TGF-B pathway have also been described in the pathogenesis of aortic aneurysms in multiple genetic disorders, including Loeys-Dietz syndrome.
• On gross pathology, diameter enlargement, atheromatous plaques (especially in AAA), and/or dissection/rupture are characteristic findings of aortic aneurysms.
• On microscopic histopathological analysis, dilation, inflammatory signs if etiology is associated with inflammatory process, thrombus and atheroma are characteristic findings of aortic aneurysms.
• Lindsay ME, Dietz HC. The genetic basis of aortic aneurysm. Cold Spring Harb Perspect Med. 2014;4(9):a015909. Published . doi:10.1101/cshperspect.a015909
• Lindsay ME, Dietz HC. Lessons on the pathogenesis of aneurysm from heritable conditions. Nature. 2011;473(7347):308-16.

Differentiating [disease name] from other Diseases

• Aortic aneurysms associated with certain high risk features and familial etiology must be differentiated from other causes as some of these aneurysms are at higher risk of rupture at earlier age.

• Aortic aneurysms should be differentiated from aortic dilation that has not reached the aneurysmal level.

Epidemiology and Demographics

• The incidence of thoracic aortic aneurysm is approximately 10 per 100,000 person-years.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop aortic aneurysms.

• Abdominal aortic aneurysm is more commonly observed among patients aged 65 years old and older.
• Thoracic aortic aneurysms are commonly observed among younger patients, especially those with familial etiology.

Gender

• Thoracic aortic aneurysm affects men and women equally.

• Males are more commonly affected with AAA than females.
• The male to female ratio is approximately 4 to 1 for AAA.
• Hannawa KK, Eliason JL, Upchurch GR. Gender differences in abdominal aortic aneurysms. Vascular. 2009;17 Suppl 1(Suppl 1):S30-9.

Race

• AAA usually affects individuals of the white race.
• Black individuals are less likely to develop AAA.
• https://www.ncbi.nlm.nih.gov/pubmed/18490563

Risk Factors

• Common risk factors in the development of aortic aneurysms are age, smoking, white race, atherosclerosis, and having underlying congenital disease that is associated with aortic aneurysms.

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].

Aneurysms that coexist in both parts of the aorta are termed thoracoabdominal aneurysms (TAA).

Related Chapters

• Aortic Dissection
• Thoracic Aortic Aneurysm
• Abdominal Aortic Aneurysm
• Aneurysm

References

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