Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery

Background This study investigates the correlation between surgical timing and 15-year longitudinal left ventricular and mitral valve function, after repair of anomalous coronary artery origin from the pulmonary artery. Methods Between 1987 and 2002, 31 patients (median age, 7.1 months) underwent repair for anomalous origin of the left (n = 28), right (n = 2), or both (n = 1) coronary arteries from the pulmonary artery. Repair was accomplished by subclavian interposition in 5 patients, intrapulmonary tunnel in 12, and direct aortic reimplantation in 14. Primary mitral valve repair was never associated with coronary revascularization. Total follow-up was 186.4 patient-years (mean, 77.2 months). Results Fifteen-year actuarial survival was 92.9% ± 4.9% for coronary transfer, 40.0% ± 21.9% for subclavian interposition, and 89.9% ± 7.5% for intrapulmonary tunnel (p = 0.019). Five patients required further intervention for supravalvular pulmonary stenosis (n = 3), baffle leak (n = 1), and mitral valve replacement (n = 1). Coronary transfer allowed best freedom from long-term reoperation (92.3% ± 7.4%). Left ventricular shortening fraction increased from 17.3% ± 6.3% before operation to 34.1% ± 4.6% at last follow-up (p< 0.01). Regression analysis demonstrated a linear relationship between age at repair and shortening fraction recovery (r2 = 0.573, p< 0.01). Patients younger than 6 months of age showed worse preoperative shortening fraction (15.9% ± 5.2%) and best longitudinal shortening fraction recovery (36.4% ± 5.1%; p< 0.001). Major improvement in mitral valve function was observed within 1 year from surgery in 90.4% of survivors. Conclusions Repair of anomalous coronary artery origin from the pulmonary artery in younger symptomatic infants offers the best potential for recovery of left ventricular function, despite a worse initial presentation. Coronary transfer is associated with superior long-term survival and freedom from reoperation. Most patients with patent two-coronary repair will recover normal mitral valve function; therefore, simultaneous mitral valve surgery seems unwarranted.