A rare cause of aortic insufficiency: quadricuspid aortic valve

Quadricuspid aortic valve can be documented incidentally as a rare cause of aortic regurgitation. An asymptomatic 24-year-old male patient underwent cardiac evaluation after detection of a cardiac murmur on routine examination. There was a grade 1/6 early diastolic murmur on the right upper side of the sternum. The electrocardiogram was in sinus rhythm and normal. Transthoracic echocardiography showed normal left ventricular dimensions and functions, but it did not provide an adequate visualization of valve structure. Color Doppler examination showed mild aortic insufficiency. Transesophageal echocardiography revealed a qaudricuspid aortic valve composed of two equal large cusps and two equal small cusps. Color Doppler scans demonstrated aortic regurgitant flow at the coaptation sites of small and large cusps. Further studies by aortic computed tomographic (CT) angiography and cardiac CT did not show aortic coarctation or any other coronary abnormality, but the latter confirmed quadricuspid aortic valve.