Surgical correction of anomalous origin of the left coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly seen in 0.26% of all congenital heart defects. From 1978 to the present, we have encountered 11 such cases in patients ranging from 3 months to 60 years of age. A variety of procedures have been performed, such as ligation of the anomalous left coronary artery (n = 1), saphenous vein bypass (n = 2), subclavian to left coronary artery anastomosis (n = 4), Takeuchi repair (n = 1), and revascularization using a 6-mm Gore-Tex tube (n = 1). Two of these patients were children who also had prosthetic mitral valve replacements with a #1 M Starr-Edwards valve and left subclavian to left coronary artery anastomosis. Three patients died postoperatively, one immediately after surgery due to low cardiac output, another on the eighth postoperative day due to renal failure, and the third was a child who died 3 months later due to bacterial endocarditis of the prosthetic valve. The surviving patients have been and are in NYHA Class I after a mean follow-up of 9 years. One patient, revascularized with a prosthetic graft, was lost for follow-up after 3 years. A two-coronary system appears more physiological and is reported to be favored by most surgeons. Left subclavian to left coronary artery anastomosis has also been observed to give excellent results, with which we also agree. The mitral valve replacement in addition to this procedure, reported here, has not been described before in the literature.