[Aortic valve replacement for aortic valve stenosis due to congenital bicuspid aortic valve with abnormal positioning of coronary orifice, pseudotendon, and persistent left superior vena cava, report of a case].

: A patient was a 65-year-old female who had a complaint of palpitation was diagnosed aortic valve stenosis due to congenital bicuspid aortic valve with pseudotendon by the echocardiographic examination. We suspected left single coronary artery by the aortography and the coronary artery angiography. Aortic valve replacement and resection of pseudotendon was performed with Carbomedics supra-annular aortic valve (21 A). During surgery, persistent left superior vena cava was detected. High-posterior take-off right coronary artery was casually detected at aortic closure. Ventricular fibrillation due to insufficient supply of cardioplegic solution at right coronary area frequently occurred after cardio-pulmonary bypass and percutaneous cardiopulmonary support was required. The patient was discharged 32 days after the operation. Preoperative and intraoperative evaluation was important in the case of aortic valvular disease.