Outcomes of Anomalous Left Coronary Artery From Pulmonary Artery Repair: Beyond Normal Function

Background Anomalous left coronary artery from pulmonary artery (ALCAPA) is associated with high mortality if left uncorrected. We describe long-term outcomes after surgical repair and ventricular function changes on long-term follow-up. Methods A retrospective review of patients who had ALCAPA repair from January 1996 to December 2011 was completed. Ventricular function was assessed by shortening fraction and ejection fraction, left ventricular end-diastolic dimension, and severity of mitral regurgitation. Speckle tracking echocardiography served as a marker of early myocardial dysfunction. Results In total, 34 patients underwent ALCAPA repair at median age of 5 months (range, 3 days to 39 years). Surgical interventions included coronary translocation in 31 patients (91%), Takeuchi repair in 2 (6%), and coronary ligation in 1 patient (3%). Concomitant mitral valve repair was performed in 5 patients (15%). No patient required mechanical circulatory support postoperatively. There was no early mortality. At median follow-up of 6 years (range, 1 month to 14 years), there were 5 reoperations: 2 heart transplants (6%), 1 mitral valve replacement (3%), 1 coronary artery bypass graft (3%), and 1 ventricular septal defect closure (3%); there was 1 death 2 years after surgery. Ejection fraction improved from 21% ± 6% to 60% ± 7% ( p = 0.008), mean shortening fraction from 25% ± 14% to 38% ± 5% ( p = 0.01), mean left ventricular end-diastolic dimension Z-score from 6.0 ± 3.8 to 0.9 ± 0.7 ( p < 0.001), and number of patients with moderate-severe mitral regurgitation from 44% (15 of 34) to 5% (1 of 21). All patients had normal ejection fraction and shortening fraction at last follow-up. Speckle tracking echocardiography showed decreased global longitudinal ( p = 0.01) and circumferential strain ( p = 0.03) for 11 of 14 patients (79%). Conclusions Excellent outcomes are achieved with repair of ALCAPA without mechanical circulatory support and with low reintervention rates. Normal ejection fraction and shortening fraction do not accurately convey myocardial dysfunction in these patients.