Thoracic aortic aneurysm screening

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]

Overview

Screening for TAA is not recommended in the general population. However, 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. First-degree relatives of people with thoracic aortic aneurysm or dissection should have aortic imaging to identify asymptomatic disease. Patients with high risk syndromes such as Marfan's syndrome and Loeys-Dietz syndrome should undergo complete aortic imaging at initial diagnosis and 6 months thereafter to establish if enlargement is occurring

Surveillance

Marfan's syndrome

People diagnosed with Marfan syndrome should immediately have an echocardiogram to measure the aorta, and followed up 6 months later to check for aortic enlargement. 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 (Level of Evidence: C)

Loeys-Dietz Syndrome

Patients with Loeys-Dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11) should undergo complete aortic imaging at initial diagnosis and 6 months thereafter to establish if enlargement is occurring.

Turner's Syndrome

Patients with Turner syndrome should undergo imaging of the heart and aorta for evidence of bicuspid aortic valve, coarctation of the aorta, or dilatation of the ascending thoracic aorta. If initial imaging is normal and there are no risk factors for aortic dissection, repeat imaging should be performed every 5 to 10 years or if otherwise clinically indicated. If abnormalities exist, annual imaging or follow-up imaging should be done.

2010 ACC/ AHA Guidelines for Aortic Imaging Techniques to determine the presence and progression of Thoracic aortic disease (DO NOT EDIT)

Class I

1. Measurements of aortic diameter should be taken at reproducible anatomic landmarks, perpendicular to the axis of blood flow, and reported in a clear and consistent format. (Level of Evidence: C)

2. For measurements taken by computed tomographic imaging or magnetic resonance imaging, the external diameter should be measured perpendicular to the axis of blood flow. For aortic root measurements, the widest diameter, typically at the mid-sinus level, should be used. (Level of Evidence: C)

3. For measurements taken by echocardiography, the internal diameter should be measured perpendicular to the axis of blood flow. For aortic root measurements the widest diameter, typically at the mid-sinus level, should be used. (Level of Evidence: C)

4. Abnormalities of aortic morphology should be recognized and reported separately even when aortic diameters are within normal limits. (Level of Evidence:C)

5. The finding of aortic dissection, aneurysm, traumatic injury and/or aortic rupture should be immediately communicated to the referring physician. (Level of Evidence:C)

6. Techniques to minimize episodic and cumulative radiation exposure should be utilized whenever possible. (Level of Evidence:)

Class IIa

1. If clinical information is available, it can be useful to relate aortic diameter to the patient's age and body size. (Level of Evidence:C)

2010 ACC/ AHA Guidelines - Recommendations for Aortic Imaging techniques in Genetic syndromes (DO NOT EDIT)

Class I

1. 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. (Level of Evidence: C)

2. 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 (Level of Evidence: C)

3. Patients with Loeys-Dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11) should undergo complete aortic imaging at initial diagnosis and 6 months thereafter to establish if enlargement is occurring (Level of Evidence: C)

4. Patients with Loeys-Dietz syndrome should have yearly magnetic resonance imaging from the cerebrovascular circulation to the pelvis (Level of Evidence:B)

5. Patients with Turner syndrome should undergo imaging of the heart and aorta for evidence of bicuspid aortic valve, coarctation of the aorta, or dilatation of the ascending thoracic aorta. If initial imaging is normal and there are no risk factors for aortic dissection, repeat imaging should be performed every 5 to 10 years or if otherwise clinically indicated. If abnormalities exist, annual imaging or follow-up imaging should be done. (Level of Evidence:C)

Class IIa

1.It is reasonable to consider surgical repair of the aorta in all adult patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation and an aortic diameter of 4.2 cm or greater by transesophageal echocardiogram (internal diameter) or 4.4 to 4.6 cm or greater by computed tomographic imaging and/or magnetic resonance imaging (external diameter). (Level of Evidence:C)

2.For women with Marfan syndrome contemplating pregnancy, it is reasonable to prophylactically replace the aortic root and ascending aorta if the diameter exceeds 4.0 cm. (Level of Evidence:C)

3. If the maximal cross-sectional area in square centimeters of the ascending aorta or root divided by the patient's height in meters exceeds a ratio of 10, surgical repair is reasonable because shorter patients have dissection at a smaller size and 15% of patients with Marfan syndrome have dissection at a size smaller than 5.0 cm. (Level of Evidence:C)

Class IIb

a. In patients with Turner syndrome with additional risk factors, including bicuspid aortic valve, coarctation of the aorta, and/or hypertension, and in patients who attempt to become pregnant or who become pregnant, it may be reasonable to perform imaging of the heart and aorta to help determine the risk of aortic dissection (Level of Evidence:C)

2010 ACC/ AHA Guidelines - Recommendations for Aortic imaging techniques for Familial thoracic aortic aneurysms and dissections (DO NOT EDIT)

Class I

1.Aortic imaging is recommended for first-degree relatives of patients with thoracic aortic aneurysm and/or dissection to identify those with asymptomatic disease (Level of Evidence: B)

2. If the mutant gene (FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2, MYH11) associated with aortic aneurysm and/or dissection is identified in a patient, first-degree relatives should undergo counseling and testing. Then, only the relatives with the genetic mutation should undergo aortic imaging. (Level of Evidence: C)

Class IIa

1.If one or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilatation, aneurysm, or dissection, then imaging of second-degree relatives is reasonable (Level of Evidence:A)

2. Sequencing of the ACTA2 gene is reasonable in patients with a family history of thoracic aortic aneurysms and/or dissections to determine if ACTA2 mutations are responsible for the inherited predisposition (Level of Evidence:B)

Class IIb

1. Sequencing of other genes known to cause familial thoracic aortic aneurysms and/or dissection (TGFBR1, TGFBR2, MYH11) may be considered in patients with a family history and clinical features associated with mutations in these genes(Level of Evidence:B)

2. If one or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilatation, aneurysm, or dissection, then referral to a geneticist may be considered.(Level of Evidence:C)

2010 ACC/ AHA Guidelines - Recommendations for Aortic imaging techniques in Bicuspid aortic valve and associated congenital variants in adults (DO NOT EDIT)

Class I

1. 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.(Level of Evidence: C)

2. All patient.s with a bicuspid aortic valve should have both the aortic root and ascending thoracic aorta evaluated for evidence of aortic dilatation (Level of Evidence: B)

2010 ACC/ AHA Guidelines - Recommendations for Aortic imaging techniques in Takayasu's arteritis and Giant cell arteritis (DO NOT EDIT)

Class I

1.Initial therapy for active Takayasu arteritis and active giant cell arteritis should be corticosteroids at a high dose (prednisone 40 to 60 mg daily at initiation or its equivalent) to reduce the active inflammatory state (Level of Evidence: B)

2. The success of treatment of patients with Takayasu arteritis and giant cell arteritis should be periodically evaluated to determine disease activity by repeated physical examination and either an erythrocyte sedimentation rate or C-reactive protein level (Level of Evidence: B)

3.Elective revascularization of patients with Takayasu arteritis and giant cell arteritis should be delayed until the acute inflammatory state is treated and quiescent (Level of Evidence: B)

4.The initial evaluation of Takayasu arteritis or giant cell arteritis should include thoracic aorta and branch vessel computed tomographic imaging or magnetic resonance imaging to investigate the possibility of aneurysm or occlusive disease in these vessels (Level of Evidence:C)

Class IIa

1.It is reasonable to treat patients with Takayasu arteritis receiving corticosteroids with an additional anti-inflammatory agent if there is evidence of progression of vascular disease, recurrence of constitutional symptoms, or re-elevation of inflammatory marker (Level of Evidence:C)