Bicuspid Aortic Valve

Bicuspid aortic valve (BAV) disease is one of the most frequent observed congenital heart abnormalities affecting 0.5-1.4% of the general population and has a 3:1 male predominance [1-4]. Although some genes have been described that found to be responsible for the abnormal valvulogenesis, little is known about BAV disease with respect to the genetic and embryolog‐ ical insight of calcification and why patients with BAV disease develop aortic valve calcifica‐ tion including severe aortic valve stenosis (AS) at an earlier age compared with degenerative tricuspid valve. BAV disease is also associated with several cardiovascular abnormalities including coarctation of the aorta (COA) and Turner Syndrome. While patients with BAV disease are often asymptomatic during the childhood, BAV disease is generally diagnosed in the adulthood using echocardiography when aortic stenosis with or without valve calcifica‐ tion, infective endocarditis, aortic regurgitation (AR), and proximal thoracic aortic dilatation are detected, often necessitating surgical intervention [5]. Patients with BAV disease require close observation before complications as heart failure and aortic dissection may occur. In this chapter, the contemporary knowledge regarding BAV disease will be discussed.