ABSTRACT
Congenital quadricuspid aortic valve is a very rare malformation. Most cases have been discovered as an incidental finding at aortic valve surgery or at autopsy. It frequently evolves to aortic regurgitation, which can manifest in adulthood and may require surgical treatment. A 66-year-old man was admitted because of dysprea. Echocardiogram revealed aortic regurgitation, mitral regurgitation, and tricuspid regurgitation. We performed aortic valve replacement, mitral annuloplasty, and tricuspid annuloplasty successfully. The aortic valve showed one large, two intermediate and one smaller cusp, which were classified as typed by Hurwitz's classification. An accessory cusp was situated between the right and left coronary cusps. No coronary abnormality was involved. The postoperative course was uneventful and he is doing well 6 months after operation.