Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Beyond Infancy: Single-Center Experience of Clinical Spectrum and Surgical Outcomes
Betül Çınar1
, Pelin Ayyıldız1, Sertaç Haydin2, Alper Güzeltaş1
1Department of Pediatric Cardiology, University of Health Sciences, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Türkiye
2Department of Pediatric Cardiovascular Surgery, University of Health Sciences, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Türkiye
Keywords: ALCAPA; congenital heart disease; coronary reimplantation; myocardial recovery.
Abstract
Objective: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare and life-threatening congenital anomaly. While it typically manifests during early infancy, some patients remain asymptomatic or present later in childhood and adolescence due to well-developed intercoronary collaterals. Therefore, the objective of this study was to evaluate the clinical spectrum, diagnostic features, and surgical outcomes of patients diagnosed with ALCAPA beyond infancy at our center.
Methods: A retrospective review was conducted on 35 patients diagnosed with ALCAPA who underwent surgical treatment at our center between January 2012 and January 2025. Out of this cohort, 8 patients diagnosed beyond infancy (older than 12 months) were enrolled in this study to evaluate their clinical spectrum and surgical revascularization outcomes.
Results: The age at diagnosis ranged from 2 to 21 years (median: 6.8 years), and all patients were female. Four patients (50%) were completely asymptomatic at presentation. Symptomatic presentations included exertional chest pain (n=1), recurrent vomiting and irritability (n=2), and sudden cardiac arrest with sustained ventricular arrhythmia (n=1). Electrocardiography (ECG) revealed characteristic ALCAPA patterns in 5 patients. Echocardiography demonstrated left ventricular (LV) and right coronary artery (RCA) dilation in all cases, with a mean RCA/aortic annulus ratio of 0.25. Baseline LV systolic dysfunction (ejection fraction: 30–45%) was observed in 4 patients. Surgical management consisted of direct coronary reimplantation (n=6) and the Takeuchi procedure (n=2). Mitral valve repair was performed in 2 patients with structural mitral anomalies. At a mean follow-up of 48 months, all patients were alive and asymptomatic, with full recovery of LV function and resolution of ECG abnormalities within 6–9 months postoperatively.
Conclusion: ALCAPA must be considered in the differential diagnosis of unexplained LV dilation or mitral regurgitation across all pediatric age groups. Extensive collateral circulation can mask typical features, leading to atypical presentations or misdiagnosis. Prompt establishment of a dual-coronary system provides a satisfactoryprognosis and facilitates complete myocardial recovery.
Cite This Article: Çınar B, Ayyıldız P, Haydin S, Güzeltaş A. Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Beyond Infancy: Single-Center Experience of Clinical Spectrum and Surgical Outcomes. Koşuyolu Heart J 2026;29(3):188–195