Congenital quadricuspid aortic valve

This case of a patient with a congenital quadricuspid aortic valve is the seventh to be reported in the world literature. Our report indicates that a quadricuspid aortic valve appears to function as well hemodynamically as a normal cuspid valve and therefore was discovered only as an incidental finding in this 67 year old man. Multiple fenestrations of the aortic cusps and an accessory right coronary artery were also noted.Review of the literature reveals that congenital malformations of the aortic and pulmonary valves are frequently associated with other cardiac abnormalities. The quadricuspid aortic and pulmonary valves do not appear to be particularly susceptible to bacterial endocarditis. The high incidence of bacterial endocarditis in the bicuspid aortic valve appears to be directly related to rheumatic involvement of this valve. The increased incidence of bacterial endocarditis in the bicuspid pulmonary valve may well be related to the coexisting ventricular septal defect.