Background: Optimal repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) relies on the creation of a dual-coronary system. If the anomalous coronary arises at a long distance from the aorta, we use various coronary extension techniques to facilitate tension-free implantation.
Methods: Thirty patients underwent ALCAPA operations using direct coronary transfer (n = 11) or coronary extension techniques (n = 19). Surgical outcomes were analyzed.
Results: Median age and weight were 5.7 months (range, 46 days to 5.45 years) and 5.35 kg (range, 3.3 to 15.9 kg). Five patients had concomitant mitral annuloplasty. Mean cardiopulmonary bypass and ischemic times were 108 +/- 38 and 57 +/- 25 minutes. Two patients required intraoperative revision of the implantation. There were three hospital deaths (10%) and no late deaths. Follow-up echocardiograms demonstrated significant improvement postoperatively vs preoperatively in shortening fraction (35% +/- 2% vs 16% +/- 2%, p < 0.00001), ejection fraction (64% +/- 3% vs 32% +/- 4%, p < 0.00001), and mitral regurgitation (11% moderate vs 70% moderate or severe, p = 0.0002). Left ventricular end-diastolic dimension Z-score decreased from 9.1 +/- 0.9 to 1.2 +/- 0.5 (p < 0.00001). Both techniques were equally effective. Two patients underwent reoperation 1 and 12 years postoperatively (coronary artery bypass grafting, 1; mitral repair with coronary angioplasty, 1). Surviving patients remain asymptomatic (p < 0.00001).
Conclusions: Dual-coronary system can be established in patients with ALCAPA. Coronary extension implantation techniques have acceptable operative mortality and excellent cardiac recovery and late survival. Although the rate of late coronary occlusion is low, continual ventricular or mitral dysfunction should trigger evaluation of persistent coronary compromise.