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Bicuspid aortic valve

Definition
BAV is a common congenital cardiac abnormality consisting of two cusps instead of three that is characterized by dilatation of the thoracic aorta.
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Pathophysiology
The exact cause of BAV is unknown; however, genetic causes (Anderson syndrome, NOTCH1 mutation) have been implicated.
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Disease course
The fusion of the aortic cusps during valvulogenesis results in the BAV, which can cause clinical manifestations of severe aortic stenosis (symptoms of dyspnea, syncope, and chest pain), AR, aortic incompetence, aortopathy leading to aneurysm and dissection, infective endocarditis to asymptomatic disease.
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Prognosis and risk of recurrence
BAV in patients with definite infective endocarditis of the native aortic valve is associated with a 9% in-hospital mortality.
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Key sources
The following summarized guidelines for the evaluation and management of bicuspid aortic valve are prepared by our editorial team based on guidelines from the American Heart Association (AHA/ACC 2022; 2021), the European Society of Cardiology (ESC/EACTS 2021), the European Society of Cardiology (ESC 2014), and the Society of Thoracic Surgeons (STS 2013).
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Guidelines

1.Screening and diagnosis

Indications for screening: obtain TTE and cardiac MRI at the time of diagnosis to evaluate for BAV, aortic root and ascending aortic dilation, aortic coarctation, and other congenital heart defects in patients with Turner syndrome.
B
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  • Screening of family relatives

2.Diagnostic investigations

Transthoracic echocardiogram: as per ACC 2022 guidelines, obtain TTE to evaluate valve morphology and function, the diameter of the aortic root and ascending aorta, and to evaluate for aortic coarctation and other associated cardiovascular defects in patients with BAV.
B

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  • CT/MRI

  • Genetic testing

3.Medical management

Beta-blockers: consider β-blockers in patients with BAV and dilated aortic root > 4.0 cm.
C

4.Nonpharmacologic interventions

Exercise restrictions: advise avoidance of isometric exercise with a high static load (such as weightlifting) in patients with any elastopathy or BAV with dilated aortic root (> 4.0 cm).
B

5.Surgical interventions

Indications for aortic replacement or repair: as per ACC 2022 guidelines, perform surgery to replace the aortic root, ascending aorta, or both, in patients with BAV and a diameter of the aortic root, ascending aorta, or both, of ≥ 5.5 cm.
B
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6.Specific circumstances

Pregnant patients: obtain aortic imaging (TTE, MRI or CT, or both as appropriate) before pregnancy to determine aortic diameters in patients with BAV with aortic dilation.
B
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7.Follow-up and surveillance

Follow-up aortic imaging: as per ACC 2022 guidelines, obtain lifelong surveillance imaging of the aortic root and ascending aorta by TTE, CT, or MRI at an interval dependent on the aortic diameter and rate of growth in patients with BAV and a diameter of the aortic root, ascending aorta, or both, of ≥ 4.0 cm,
B
including patients undergone previous aortic valve repair or replacement.
B

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  • Pharmacotherapy after valve repair or replacement