Background .– Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a coronary abnormality which can be associated to other congenital heart defects which complicates the positive diagnosis of the coronary abnormality, especially before surgery.

Objective .– Here, we report a series of 13 patients with ALCAPA with a focus on the type of associated heart defect, the moment of diagnosis of the coronary abnormality related to surgery and their outcome.

Methods .– Retrospective assessment of medical files of all patients with ALCAPA and other congenital heart defects in two important French Departments of Pediatric Cardiac Surgery from 1987 to 2012.

Results .– The heart defect most frequently encountered in association to ALCAPA was aortic coarctation ( n = 5) followed by tetralogy of Fallot with or without pulmonary atresia ( n = 3). There was one case of hypoplastic left heart syndrome, one aortic atresia, one right aortic arch, one congenital mitral malformation and one infant with divided left atrium and partial anomalous pulmonary venous return of the right lung. Only four patients had a complete diagnosis of the cardiac defect and the coronary abnormality before surgery. In three cases the coronary anomaly was discovered during surgery conducted for another cardiac defect and treated at the same time by coronary reimplantation. The six remaining patients were diagnosed after cardiac repair. Three of these patients only had a post-mortem diagnosis. Eight of 13 patients died after surgery. Three of them deceased within the first 30 days after repair. The remaining patients are in good health with a median follow-up of 5.3 years (range: 2.1–8.5 years).

Discussion .– This series confirms that ALCAPA associated with other cardiac defects is often unrecognized before surgery. Pulmonary hypertension due to left-to-right shunt or coarctation can maintain an anterograde flow in the anomalous coronary artery until cardiac repair. Myocardial ischemia will only become apparent once the defect has been repaired when pulmonary pressure lowers. In this series postoperative survival was compromised mostly due to complications occurring after complex or repeated cardiac surgery.