Anomalous origin of the left coronary artery from the pulmonary artery. Early results with direct aortic reimplantation.

Between January 1991 and June 1993, eleven children with anomalous origin of the left coronary artery from the pulmonary artery underwent direct aortic reimplantation of the left coronary artery at the German Heart Institute Berlin. The patients' ages ranged from 2.5 months to 10.5 years; six were infants. Three infants were intubated and their lungs ventilated before the operation, and one was resuscitated 2 days before the operation. The electrocardiograms of eight patients indicated deep Q waves. All but three of these patients had insufficient collaterals between the right and left coronary arteries. The entire group exhibited reduced left ventricular ejection fraction (minimum 15%) including mitral valve incompetence, which was moderate in six patients and severe in three. All six infants underwent emergency operations, and the remaining children, who were older, underwent elective operations involving moderate hypothermic perfusion and cold crystalloid cardioplegia. Aortic cross-clamping time ranged from 22 to 79 minutes (mean 54 minutes). A two-coronary artery system was established in all patients by direct reimplantation of the anomalous left coronary artery into the ascending aorta. Three patients who also exhibited severe mitral valve incompetence underwent modified Kay mitral valve annuloplasty. A delayed sternal closure procedure (closure performed 1 to 10 days after the operation) was used on eight patients. A 10-month-old patient was successfully treated after the operation with a centrifugal left heart assist device and a 9-year-old patient received extracorporeal membrane oxygenation because of severe heart failure. No postoperative deaths occurred. Left ventricular end-diastolic volume decreased dramatically after the operation and returned to near normal values 1 to 9 months postoperatively. At the same time, the preoperatively depressed left ventricular ejection fraction returned to normal and mitral valve incompetence decreased or vanished in eight patients. Color Doppler echocardiograms (eleven patients) and coronary angiograms (three patients) indicated that the reimplanted left coronary artery was patent in all eleven patients during the follow-up period. Reimplantation of the left coronary artery into the ascending aorta is an effective method of establishing a two-coronary artery system in children with anomalous origin of the left coronary artery from the pulmonary artery. Mitral valve annuloplasty is recommended for patients who also have severe mitral valve incompetence. Prolonged assisted circulation must be used in cases of severe postoperative heart failure.