Congenital quadricuspid aortic valve.

Our patient was a 53-year-old man referred for coronary revascularization and a history of a heart murmur. Intraoperative transesophageal echocardiography demonstrated a quadricuspid aortic valve (Fig 1) with opening of all four leaflets during systole (Fig 2). Severe aortic insufficiency was detected with color flow Doppler (Fig 3). An aortic valve homograph was used to replace the defective valve (Fig 4—from surgeon’s perspective). No other coronary or cardiac structural abnormalities were found. The extreme rarity of quadricuspid aortic valves has been documented in both large autopsy [1] and echocardiography studies [2] with an estimated incidence of between 0.008% and 0.013% with overall fewer than 100 cases reported in the literature. Although the risks for endocarditis are unknown, patients with quadricuspid aortic valves should be followed closely and operative repair considered for those with symptoms secondary to valvular insufficiency. A thorough investigation for other cardiac abnormalities is indicated since quadricuspid aortic valves have also been encountered in patients with other cardiac abnormalities such as coronary artery anomalies, hypertrophic cardiomyopathy, and defects of the other cardiac valves [3].