TY - CHAP M1 - Book, Section TI - Bicuspid Aortic Valve Disease A1 - Lakdawala, Neal K. A1 - Sparks, Elizabeth A. A1 - Opotowsky, Alexander R. A2 - Murray, Michael F. A2 - Babyatsky, Mark W. A2 - Giovanni, Monica A. A2 - Alkuraya, Fowzan S. A2 - Stewart, Douglas R. Y1 - 2014 N1 - T2 - Clinical Genomics: Practical Applications in Adult Patient Care AB - Disease summary:Bicuspid aortic valve (BAV) disease is the commonest congenital heart lesion, affecting approximately 1% to 2% of the population and is approximately three times more common in men.BAV is typically nonsyndromic, but is seen with increased frequency in the Turner and potentially Loeys-Dietz syndromes.BAV is usually an isolated cardiac lesion but is often seen in conjunction with coarctation of the aorta and other obstructive left-sided lesions (Shone complex, hypoplastic left heart syndrome).Aortic stenosis (AS) develops in most patients with BAV. The incidence of AS increases with age and BAV is the commonest indication for aortic valve replacement in patients under 70 years old.Aortic regurgitation (AI) and infective endocarditis are also more prevalent in BAV.BAV is associated with an elevated risk of developing thoracic aortic dilation and aneurysms (TADA) and subsequent aortic dissection and rupture. In children, the risk of aortic aneurysm increases with age, indicating the progressive nature of aortic dilation.Longitudinal echocardiographic monitoring is recommended for patients with BAV, both for development or progression of valvular stenosis or regurgitation, and also for ascending aortic dilation. If the ascending aorta cannot be well visualized with transthoracic echocardiography (TTE), either computed tomography (CT) or magnetic resonance angiography (MRA) should be performed. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/12 UR - accessmedicine.mhmedical.com/content.aspx?aid=1102699531 ER -