Age-Stratified Presentation and Outcomes Following Repair of Anomalous Left Coronary Artery From the Pulmonary Artery
Seth E M Wolf1,2, Haoran Jiang3, Rebekah R Boyd1,2
1Department of Surgery, Duke Congenital Heart Surgery Research and Training Lab, Durham, NC, USA.
Abstract:
IntroductionAnomalous origin of the left coronary artery from the pulmonary artery is a rare congenital coronary anomaly that causes myocardial ischemia and heart failure. Although surgical repair has favorable outcomes, age-related differences in presentation and recovery are not well defined.MethodsWe performed a retrospective review of patients who underwent surgical repair at a single institution between 2007 and 2023. Patients were stratified by age at repair into infants, children, and adults. Clinical presentation, operative strategy, ventricular function, mitral valve regurgitation, and survival were evaluated with longitudinal follow-up.ResultsTwenty-five patients were included, consisting of 16 infants, 5 children, and 4 adults. Infants presented with more acute disease, including severe ventricular dysfunction, ventricular dilation, mitral valve regurgitation, and preoperative instability. Children and adults more often presented incidentally or with chronic symptoms and preserved ventricular function. Coronary reimplantation was performed in nearly all patients. Infants demonstrated marked improvement in ventricular function and regression of ventricular dilation during follow-up. Mitral valve regurgitation improved in most patients following reimplantation, with selective mitral valve repair reserved for severe cases. There was one early death of an infant with profound preoperative compromise. Long-term survival was excellent, and late cardiac reintervention was uncommon.ConclusionSurgical repair results in excellent long-term outcomes across all age groups. Infants present with more severe myocardial dysfunction but demonstrate substantial ventricular recovery following revascularization, while older patients exhibit a distinct clinical phenotype with preserved ventricular function.
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