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 ori gin of the left coronary artery from the pulmonary artery underwent di rect aortic reimplantation of the left coronary artery at the German H eart Institute Berlin. The patients' ages ranged from 2.5 months to 10 .5 years; six were infants. Three infants were intubated and their lun gs ventilated before the operation, and one was resuscitated 2 days be fore the operation. The electrocardiograms of eight patients indicated deep Q waves. All but three of these patients had insufficient collat erals between the right and left coronary arteries. The entire group e xhibited reduced left ventricular ejection fraction (minimum 15%) incl uding mitral valve incompetence, which was moderate in six patients an d 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 cardiop legia. Aortic crossclamping time ranged from 22 to 79 minutes (mean 54 minutes). A two-coronary artery system was established in all patient s by direct reimplantation of the anomalous left coronary artery into the ascending aorta. Three patients who also exhibited severe mitral v alve incompetence underwent modified Kay mitral valve annuloplasty. A delayed sternal closure procedure (closure performed 1 to 10 days afte r the operation) was used on eight patients. A 10-month-old patient wa s successfully treated after the operation with a centrifugal left hea rt assist device and a 9-year-old patient received extracorporeal memb rane oxygenation because of severe heart failure. No postoperative dea ths occurred. Left ventricular end-diastolic volume decreased dramatic ally after the operation and returned to near normal values 1 to 9 mon ths postoperatively. At the same time, the preoperatively depressed le ft ventricular ejection fraction returned to normal and mitral valve i ncompetence decreased or vanished in eight patients. Color Doppler ech ocardiograms (eleven patients) and coronary angiograms (three patients ) indicated that the reimplanted left coronary artery was patent in al l eleven patients during the follow-up period. Reimplantation of the l eft 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 v alve annuloplasty is recommended for patients who also have severe mit ral valve incompetence. Prolonged assisted circulation must be used in cases of severe postoperative heart failure.