Quadricuspid pulmonary valve identified by transthoracic echocardiography

The quadricuspid pulmonary valve is a rare congenital anomaly. It tends to be clinically quiescent. Its diagnosis by two-dimensional echocardiography could be very difficult because of the anatomical features. We report on the echocardiographic findings of a 66-year-old female patient with mitral and aortic regurgitation of rheumatic origin and severe pulmonary hypertension. There was an aneurismatic dilation of the pulmonary artery trunk allowing visualization of the short-axis view of the pulmonary valve. It showed four cusps of similar size and an important deficit of central coaptation. Very few cases of the quadricuspid pulmonary valve are documented in live patients.