Congenital combined atresia of the left main coronary with supravalvar aortic stenosis.

Congenital atresia of the left main coronary artery (LMCA) is an exceedingly rare phenomenon, and in the most of them, coronary artery bypass graft is required. We here describe a rare case of this anomaly that concomitantly was associated with supravalvar aortic stenosis and coronary-pulmonary fistula without the presence of conventional collateral circulation in a 16-year-old boy. The patient was admitted to our center with chest pain and dyspnea. Echocardiographic examinations showed supravalvar aortic stenosis with normal function of the aortic valve. Coronary angiography revealed atresia of LMCA with poorly developed left anterior descending coronary artery and well-developed circumflex coronary artery and diagonal artery that perfused by dominant and lengthy right coronary artery. The patient underwent coronary artery bypass grafting with repair of supravalvar aortic stenosis. The postoperative course was uneventful. The 6-month follow-up revealed normal diameter of the ascending aorta with symptomatic relief of preoperative chest complaint.