Bicuspid aortic valve disease is the most common congenital cardiac anomaly. The prevalence in the general population is between 0.46% and 1.37%. There is significantly high cardiac morbidity associated with bicuspid aortic valve disease, predominantly due to progressive valve dysfunction (stenosis or regurgitation) that requires surgical intervention for symptom relief or prevention of left ventricular dysfunction, or less commonly, for complications of endocarditis. Bicuspid aortic valve disease is clinically important not only because of valve disease but also because of its association with many vascular abnormalities, such as aortic dilatation and aortic coarctation.