Anomalous origin of the left coronary artery: a review of surgical management in 13 patients.

Over the last decades, surgical management of anomalous left coronary artery originating from the pulmonary artery (ALCAPA) has seen a considerable evolution. Between 1965 and 1992, 13 children with age at operation ranging between 2 months and 11 years (mean 23 months) underwent one of 5 different surgical procedures: ligation (n = 3), direct aortic reimplantation (n = 7), subclavian (n = 1) or internal mammary (n = 1) artery anastomosis, or modified aortic implantation (Vigneswaran-procedure, n = 1). There were 3 early and one late deaths resulting in an overall mortality of 30.7%. Mortality was 66.7% in the ligation group, and 20% in the revascularization group (28.6% in the subgroup with direct aortic implantation). The one late death occurred 6 months after ligation. Follow-up ranges between 3 months and 21 years, mean 7.3 years. All but one survivors are in NYHA functional class I. Following operative correction, there was clear improvement in left ventricular performance. Our data give reason to suggest ALCAPA to be more frequent than considered so far. Early surgery is recommended in all patients with ALCAPA, regardless of age or symptomatic status. Reestablishment of a two-coronary system is considered the procedure of choice. All survivors require long-term follow-up controls of early recognition of residual or progressive cardiac problems.