Congenital left main coronary artery to main pulmonary artery fistula with bicuspid aortic valve: a case report and review of literature.

Congenital coronary artery fistulas (CAFs) are uncommon abnormalities. A connection between the left main stem and main pulmonary artery is extremely rare. Congenital CAFs are frequently associated with another congenital heart disease. Associated anomalies include atrial septal defect, tetralogy of Fallot, patent ductus arteriosus, ventricular septal defect, and pulmonary atresia. Association of bicuspid aortic valve with CAF has not been reported in literature to date. We report on a 68-year-old man with congenital left main to pulmonary artery fistula associated with bicuspid aortic valve and moderate aortic stenoses, who underwent successful aortic valve replacement with ligation of CAF and also review the natural history, pathophysiology, and management of CAF.