Key result
Surgical repair for ALCAPA syndrome resulted in an overall survival of 93.4% at 5 years and 83.6% at 10 years, though the need for reintervention increased over time.
Why the study?
Data on long-term sequelae after surgical correction of anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome are minimal.
What are the long-term survival and functional outcomes following surgical repair of ALCAPA syndrome in pediatric and adult patients?
Cohort (n=31)
Yes
What are the long-term survival and functional outcomes following surgical repair of ALCAPA syndrome in pediatric and adult patients?
Answers are grounded in this paper and cite their sources.
Surgical repair of ALCAPA syndrome yields excellent long-term survival and improves LVEF, though reintervention rates increase over time and significant mitral regurgitation often persists in patients diagnosed as adults.
Background: 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. Methods: All patients with ALCAPA seen within our hospital system from 1965 to 2022 were reviewed. Patients with other structural heart diseases were excluded. Results: 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. Conclusion: 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.
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Smith et al. (2025) conducted a cohort in Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome (n=31). Surgical repair was evaluated on Overall survival at 10 years (95% CI 69.8-100). Surgical repair for ALCAPA syndrome resulted in an overall survival of 93.4% at 5 years and 83.6% at 10 years, though the need for reintervention increased over time.
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