Anomalous left coronary artery from the pulmonary artery in infants. Which operation? When?

The surgical management of anomalous left coronary artery from the pulmonary artery in infants and small children remains controversial, because the ideal surgical procedure and the optimal time for operation are yet to be determined. From 1977 to 1985, 22 patients less than 4 years of age (mean age 18.2 months) underwent direct aortic reimplantation of the anomalous left coronary artery. There were five operative deaths (23%, confidence limits 13%-36%). The determinant risk factor of early mortality was the severity of preoperative left ventricular dysfunction (p = 0.05), not age at operation (p = 0.64) or preoperative clinical status (p = 0.36). There were not late deaths (mean follow-up 38 months). All survivors but one were symptom free. The reimplanted anomalous left coronary artery was patent in each reevaluated case (9/17). Left ventricular function improved significantly in all survivors. Moderate to severe preoperative mitral incompetence lessened in all patients but one, without mitral valve repair. When technically feasible, direct aortic reimplantation of the anomalous left coronary artery is an attractive procedure because it offers a high rate of patency and avoids the potential drawbacks of procedures involving autogenous venous or arterial tissue. Optimal intraoperative myocardial preservation and institution of temporary left ventricular assistance at the end of the operation may decrease the operative risk. Left ventricular function nearly always recovers after successful revascularization, and resection of left ventricular myocardium is rarely indicated, if ever. Mitral incompetence almost always lessens, and the mitral valve should not be repaired at initial operation; however, residual mitral incompetence may necessitate reoperation in a few cases. In infants with moderate left ventricular damage (usually asymptomatic with medical therapy), surgical treatment should be delayed until 18 to 24 months of age so that it can be performed with a low operative risk. Infants with severely impaired left ventricular function and persistent congestive heart failure should probably undergo operation as soon as the diagnosis has been made.