Long-term Outcomes in Anomalous Left Coronary Artery From the Pulmonary Artery Syndrome: A Multicenter Half-Century...

Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a congenital anomaly that presents in infancy or rarely in adulthood and generally requires surgical correction. Data on long-term sequelae are minimal.

All patients with ALCAPA seen within our hospital system from 1965 to 2022 were reviewed. Patients with other structural heart diseases were excluded.

Thirty-one patients were identified, with 9 patients (29.0%) being diagnosed as adults. The cohort was divided into pediatric (<18 years) and adult (≥18 years) subgroups. Heart failure was the most common presentation in pediatric patients (36.4%), whereas dyspnea and chest pain were more common in adults (44.4% and 33.3%, respectively). All patients underwent surgical repair. Overall survival was 93.4% at 1 year and 5 years (95% confidence interval [CI]: 85-100) and 83.6% at 10 years (95% CI: 69.8-100). The need for reintervention was 0% at 1 year, 15.8% at 5 years (95% CI: 0-31), and 29.8% at 10 years (95% CI: 3.4-49). Preoperative ejection fraction (left ventricular ejection fraction [LVEF]) was 51% for pediatric patients and 43% for adults; follow-up LVEF was 61% among pediatric patients and 55% among adults. Significant mitral valve regurgitation (MR) was noted on the preoperative echocardiogram in 30.8% of pediatric patients and 42.9% of adults. The prevalence of MR decreased to 11.8% in pediatric patients, but it was still 40% in adults at long-term follow-up.

ALCAPA has variable presentations in childhood and adulthood. Overall survival after surgery is excellent. LVEF improved throughout the cohort and MR improved in pediatric patients. Reintervention rates increased over time.