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Bicuspid aortic valve
Background
Overview
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), aortic regurgitation, 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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Guidelines
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 European Society of Cardiology (ESC 2024,2014), the American Heart Association (AHA/ACC 2022,2021), the European Society of Cardiology (ESC/EACTS 2022), and the Society of Thoracic Surgeons (STS 2013).
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Screening and diagnosis
Indications for screening: as per ACC/AHA 2022 guidelines, 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.
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Screening of family relatives
Diagnostic investigations
TTE: as per ESC 2024 guidelines, obtain an initial TTE to assess diameters of the aorta at several levels when a BAV is first diagnosed.
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CT/MRI
Genetic testing
Medical management
Nonpharmacologic interventions
Surgical interventions
Specific circumstances
Follow-up and surveillance
Follow-up aortic imaging
As per ESC 2024 guidelines:
Obtain surveillance serial imaging by TTE in patients with BAV with a maximum aortic diameter > 40 mm, either with no indication for surgery or after isolated aortic valve surgery, after 1 year, then if stable, every 2-3 years.
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Obtain cardiovascular CT or cardiovascular MRI of the entire thoracic aorta when important discrepancies in measurements are found between subsequent TTE controls during surveillance.
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Pharmacotherapy after valve repair or replacement