Abstract
Quadricuspid aortic valve, like bicuspid aortic valve, is characterized by congenital malformation and resulting dysfunction of the aortic valve. This condition is rare, occurring less frequently than bicuspid aortic valve. We report two cases of quadricuspid aortic valve diagnosed preoperatively by echocardiography. Case 1: Quadricuspid aortic valve in a 63-year-old man who had previously received a permanent pacemaker. The patient presented dyspnea on exertion and was hospitalized. Echocardiography showed severe aortic regurgitation in a four-cuspid aortic valve with an accessory cusp between the left and non-coronary cusps. Quadricuspid aortic valve was confirmed on surgery, and the valve was replaced with a prosthesis (SJM 23 mm). Mild fibrosis and myxomatous degeneration were observed on histologic examination. Case 2: Quadricuspid aortic valve in a 58-year-old woman under treatment for hypertension and bronchial asthma. The patient presented dyspnea on exertion and was hospitalized. Transthoracic echocardiography showed severe aortic regurgitation and aortic stenosis, but the configuration of the aortic valve was unclear. Transesophageal echocardiography demonstrated a four-cuspid aortic valve with an accessory cusp between the left and right coronary cusps. Quadricuspid aortic valve was confirmed on surgery, and the valve was replaced with a prosthesis (SJM 17 mm). Histologic examination showed calcification and extensive fibrosis. Quadricuspid aortic valve should be considered when using echocardiography to investigate the cause of aortic valve disease.
