Bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with other valvular abnormalities and aortopathies. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Patients require regular surveillance for progressive valvular disease and aortopathy. Although bicuspid aortic valve may occur sporadically, many cases are inherited, so first-degree relatives should be evaluated and genetic counseling considered.
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