Thoracic aortic disease pathophysiology
Overview
2010 ACCF/AHA Guideline Recommendations: Genetic Syndromes associated with Thoracic Aortic Aneurysms and Dissection (DO NOT EDIT) [1]
Recommendations for Genetic Syndromes
| 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 IIb |
| "1. 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)" |
Recommendations for Genetic Syndromes of Familial Thoracic Aortic Aneurysms and Dissections
| 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: B)" |
| "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)" |
ACC/AHA Guidelines - Guidelines for other Cardiovascular conditions associated with Thoracic aortic aneurysm and dissection (DO NOT EDIT) [1]
Recommendations for Bicuspid aortic valve and associated Congenital variants in adults
| 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 patients 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)" |
ACC/AHA Guidelines - Guidelines for Inflammatory diseases associated with Thoracic aortic disease (DO NOT EDIT) [1]
Recommendations for Takayasu Arteritis and Giant Cell Arteritis
| 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 additionalanti-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) " |
ACC/AHA Guidelines - Guidelines for Aortic arch and Thoracic aortic atheroma and atheroembolic disease. (DO NOT EDIT) [1]
Recommendations for Aortic arch and Thoracic aortic atheroma and atheroembolic disease
| Class IIa |
| "1. Treatment with a statin is a reasonable option for patients with aortic arch atheroma to reduce the risk of stroke. (Level of Evidence: C)" |
| Class IIb |
| "1. Oral anticoagulation therapy with warfarin (INR 2.0 to 3.0) or antiplatelet therapy may be considered in stroke patients with aortic arch atheroma 4.0 mm or greater to prevent recurrent stroke. (Level of Evidence: C)" |
References
- ↑ 1.0 1.1 1.2 1.3 Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE; et al. (2010). "2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine". Circulation. 121 (13): e266–369. doi:10.1161/CIR.0b013e3181d4739e. PMID 20233780.