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thoracic aortic aneurysm

Etiology: - atherosclerosis - *connective tissue disease - Marfan syndrome - Ehlers-Danlos syndrome type 4 - Loeys-Dietz syndrome - *genetic disease/congenital disorder - TAAD syndrome - bicuspid aortic valve - Turner syndrome - family history of thoracic aortic aneurysm or aortic dissection - *vasculitis - Takayasu arteritis - giant cell arteritis - aortitis - Behcet syndrome - systemic lupus erythematosus - *infections - septic embolism - *direct bacterial innoculation - *bacterial seeding - *contiguous infection - syphilis - *aortic injury - prior acute aortic syndrome - thoracic traums Epidemiology: see aortic aneurysm Clinical manifestations: - chest pain, abdominal pain, flank pain, back pain - murmur of aortic insufficiency - heart failure - blood pressure differential between arms - hoarseness, dysphagia, recurrent pneumonia, superior vena cava syndrome Laboratory: - plasma D-dimer < 500 ug/L within 1st 24 hours of symptom onset with 95% negative predictive value [3] Special laboratory: - echocardiogram - follow-up measurement of aortic root diameter - yearly, until aortic root >= 4.5 cm, then every 6 months - aortic root > 5.5 cm (general threshold for surgery) - aortic root > 4.5 cm (with other heart surgery) - aortic root growth > 0.5 cm/year - different schedule for Marfan syndrome & Loeys-Dietz syndrome - aortic root > 4.0-5.0 cm - screening asymptomatic patients for thoracic aortic aneurysm reserved for patients with cystic medial degeneration (Marfan's syndrome, Ehlers-Danlos syndrome), bicuspid aortic valve, or family history of aortic disease (including 1st degree relatives of patient with bicuspid aortic valve) [1] - bedside transesophageal echocardiogram (diagnostic) - critically ill patients - need for continuous monitoring Radiology: - chest X-ray may show widened mediastinum - computed tomography (CT) with contrast (diagnostic) - CT angiography (ACR) - magnetic resonance imaging (diagnostic) - magnetic resonance angiography (ACR) - with familial form, CT or MRI baseline, at 6 months, then annually if aortic size is stable [1] - imaging of thoracic aorta with angiography reserved for patients in whom percutaneous intervention is planned [1] Complications: - rupture of aneurysm - aortic dissection - thrombolism, ischemic stroke - 2-5-8% with endovascular repair - myocardial infarction - cardiac tamponade Management: 1) see aortic aneurysm 2) advise against pregnancy if > 4 cm 3) monitor aortic root diameter: see echocardiogram 4) surgical repair (aortic aneurysm repair) a) indications 1] aortic root > 5.5 cm; - 4.5-5.0 cm if due to Marfan's syndrome, Ehlers-Danlos syndrome or familial form of thoracic aortic aneurysm [1] - threshold for Loeys-Dietz syndrome is 4.5 cm 2] descending thoracic aorta > 6.0 cm [1] 3] rapid rate of growth: > 0.5 cm/year [1] 4] aortic dissection 5] symptoms (hoarseness, dysphagia, back pain) b) endovascular repair & grafting - not recommended unless prohibitive surgical risk - no need for anticoagulation - prophylaxis for bacterial endocarditis not needed 4) patients with Marfan's syndrome should be evaluated with echocardiogram, immediately after diagnosis & then in 6 months to assess progression [2] 5) patient education - 1st-degree relatives of patients with thoracic aortic aneurysm or thoracic aortic dissection should undergo aortic imaging to identify asymptomatic disease. - smoking cessation

Interactions

disease interactions

Related

dissecting aortic aneurysm; aortic dissection (acute aortic syndrome) endovascular graft (endovascular repair) intramural aortic hematoma

Specific

ascending aortic aneurysm; aortic root dilation

General

aortic aneurysm

Database Correlations

OMIM correlations MORBIDMAP 120180

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 14, 15, 16. 17, 18, 19. American College of Physicians, Philadelphia 2006, 2009, 2012, 2015, 2018, 2022
  2. Hiratzka LF et al 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease. J Am Coll Cardiol. 2010 Apr 6;55(14):e27-e129 PMID: 20359588 http://content.onlinejacc.org/cgi/reprint/j.jacc.2010.02.015v1.pdf (corresponding NGC guideline withdrawn Nov 2015) - Hiratzka LF et al 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients with Thoracic Aortic Disease. Executive Summary J Am Coll Cardiol. 2010 Apr 6;55(14):1509-44 PMID: 20359609 http://content.onlinejacc.org/cgi/reprint/j.jacc.2010.02.010v1.pdf (corresponding NGC guideline withdrawn Nov 2015)
  3. Suzuki T, Distante A, Zizza A et al Diagnosis of acute aortic dissection by D-dimer: the International Registry of Acute Aortic Dissection Substudy on Biomarkers (IRAD-Bio) experience. Circulation. 2009 May 26;119(20):2702-7 PMID: 19433758
  4. Isselbacher EM, Preventza O, Hamilton Black J 3rd et al 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Dec 13;146(24):e334-e482. PMID: 36322642 PMCID: PMC9876736 (available on 2023-12-13)