Surgery for aortic valvular disease with congenital quadricuspid aortic valve

: A case of quadricuspid aortic valve is reported. A 69-year-old man was hospitalized with chest oppression at rest and abnormal electrocardiogram and diagnosed aortic regurgitation by echocardiography and aortic angiography. Aortic regurgitation was grade III according to Seller. Aortic valve replacement was performed successfully with a 21 mm St. Jude Medical valve. The aortic valve showed four cusps consisting of two equal larger cusps and two equal smaller cusps which was type C according to Hurwitz. Each valve was thickened and adhered, and fenestrations were found at each commissure. The right coronary ostium was small but not displaced. Twenty five cases in literature which were corrected surgically are also reviewed. Quadricuspid aortic valve is a rare anomaly but must be considered as a malformation which leads to severe valve failure in later life.