Thoracic aortic aneurysm resident survival guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Synonyms and keywords:

Overview

• Thoracic aortic aneurysm is a permanent localized thoracic aortic dilatation that has at least a 50% diameter increase and three aortic walls. its shape could be fusiform or saccular.
• Classification:
• Above the ligamentum arteriosum, the disease is not related to typical arterial risk factors and has a smooth, noncalcified wall accompanied by no debris or clot.
  • Aortic root aneurysm(60% of TAA occurs in the aortic root and/or ascending aorta)
  • Ascending aortic aneurysm
  • Aortic arch aneurysm(10% occur in the aortic arch)
  • Descending aortic aneurysm (at the level of the ligamentum arteriosum, just distal to the origin of the subclavian artery,40% of TAA occur in the descending aorta), the disease process primarily is atherosclerotic, with an irregular calcified wall accompanied by copious debris and clot
  • thoracoabdominal aorta (10% of TAA occur in the thoracoabdominal aorta, based on Crawford classification, there are 5 types of thoracoabdominal aortic aneurysm: I II III IV V)
• Symptoms

patients are usually asymptomatic and diagnosed during imaging studies for another diagnostic reason. If its symptomatic, symptoms could be due to aneurysmal dissection, rupture, or bony erosion, or due to mass effect from a large thoracic aortic aneurysm, presents with Hoarseness (recurrent laryngeal nerve), dyspnea (trachea, mainstem bronchus, pulmonary artery), central venous hypertension (superior vena cava syndrome), dysphagia (esophagus), Rupture of thoracic aortic aneurysms (rupture of an ascending aortic aneurysm may cause cardiac tamponade, a rupture in the descending thoracic aorta may cause hemothorax, aortobronchial fistula, or aortoesophageal fistula)

• Evaluation can be done by CT, MRI, and TEE, PET scan, or with a CXR which may show a convex contour of the right superior mediastinum, indicating an aortic aneurysm.

Causes

Life Threatening Causes

• Aortic dissection

Common Causes

• Causes:
  • Arteriolosclerosis &Hypertension
  • Hyperlipidemia
  • Aging
  • Smoking
    
  • Genetic inheritance(with mutations in genes such as FBN1, TGFBR1, TGFBR2, ACTA2, and MYH11)
    
  • Inflammatory causes(Syphilis,Mycotic aneurysm from endocarditis,Giant-cell arteritis,Takayasu arteritis)
    
  • Trauma
  • Connective tissue; Marfan’s syndrome, Loeys-Dietz syndrome, Ehlers Danlos syndrome (vascular form) ,other connective tissue diseases, Turner syndrome
  • Known aortic valve disease e.g.Bicuspid AV
  • Family history of aortic disease
  • Recent aortic manipulation (surgical or catheter-based)

Screening

Screening for AA in certain population:

certain population substrates, such as those with history of marfan's syndrome, turner's syndrome, ehlers-danlos type IV syndrome, familial thoracic aortic disease syndromes, bicuspid aortic valve, takayasu arteritis, giant cell arteritis, loeys-dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11) should have imaging study to screen for TAAs. [Applying classification of recommendations and level of evidence classification( ACC AHA guidelines classification scheme)]

Screening for AA in patients with Marfan's syndrome

Screening for AA in Marfan's syndrome
An echocardiogram is recommended at the time of diagnosis of Marfan syndrome to determine the aortic root and ascending aortic diameters and 6 months thereafter to determine the rate of enlargement of the aorta.(Class I, Level of Evidence: C)
Annual imaging is recommended for patients with Marfan syndrome if stability of the aortic diameter is documented.
If the maximal aortic diameter is 4.5 cm or greater, or if the aortic diameter shows significant growth from baseline, more frequent imaging should be considered.

Screening for AA in Loeys-Dietz syndrome or Confirmed genetic mutations

Screening for AA in patients with Loeys-Dietz syndrome
Patients with Loeys-Dietz syndrome should undergo complete aortic imaging at the time of diagnosis and 6 months thereafter to establish if enlargement is happening. (Class I, Level of Evidence: C). Patients with Loeys-Dietz syndrome should have yearly magnetic resonance imaging from the cerebrovascular circulation to the pelvis. ( Class I, Level of Evidence: B)

Screening for AA in patients with Turner syndrome

Screening for AA in patients with Turner syndrome
patients with Turner syndrome should undergo imaging of heart and aorta at the initial diagnosis.
If the initial imaging is normal and there are not any risk factors for aortic dissection, repeat imaging is performed every 5–10 years.(Class I, Level of Evidence: C) Annual imaging is recommended if abnormalities exist
patients with Turner syndrome, and additional risk factors (bicuspid aortic valve, coarctation of the aorta, pregnant or desiring pregnancy, and/or hypertension), it may be reasonable to perform imaging of the heart and aorta to help determine the risk of aortic dissection. (Class I, Level of Evidence: C)

Screening of family members of patients with AA or genetic mutations

Screening for AA in relatives and genetic mutations
First-degree relatives of patients with thoracic aortic aneurysm or dissection should undergo aortic imaging.(Class I,Level of Evidence: C). If thoracic aortic aneurysm or dissection found in one or more first-degree relatives, then imaging of second-degree relatives is reasonable. Also, then referral to a geneticist may be considered reasonable (Class IIa, Level of Evidence: B) to screen for the following aneurysm/dissection-associated genes: FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2, and MYH11 reasonable. (Class I,Level of Evidence: C)
Sequencing of the ACTA2 gene will determine if ACTA2 mutations are responsible for the inherited predisposition reasonable. Also,maybe sequencing of these additional genes: TGFBR1, TGFBR2, and MYH11.(Class IIa, Level of Evidence: B)
first-degree relatives of patients with a bicuspid aortic valve, premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of thoracic aortic aneurysm and dissection should be evaluated for the presence of a bicuspid aortic valve and asymptomatic thoracic aortic disease. (Class I, Level of Evidence: C)both the root and ascending aorta needs to be evaluated in patients with bicuspid aortic valves. (Class I, Level of Evidence: B)

Treatment

• Once aortic dilation is suspected, based on echocardiography and/or chest X-ray, it is reasonable to obtain definitive aortic imaging with CT or magnetic resonance angiography. If the aneurysm is too small to justify surgery, non-surgical medical therapy is recommended, then repeat imaging at six months to document stability. If the aortic dimensions remain stable, annual follow-up with CT or MRA is reasonable. If a significant size has been reached or growth has been documented, they may elect to undergo surgical repair. however, it is beneficial to have early discussions with the potential surgical candidate about the area of concern and the types of operations available, their outcomes, and associated risks and benefits.

• patients with thoracic AA must be counseled about smoking cessation.
• Dyslipidemia must be treated. Atorvastatin 40 to 80 mg daily or rosuvastatin 20 to 40 mg daily should be administered
• Hypertension must be controlled with beta-blocker plus an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker these in these patients. The blood pressure in persons with thoracic AA should be lowered to less than 130/80 mmHg.

Do's

• The content in this section is in bullet points.

Don'ts

• The content in this section is in bullet points.