The outcome of surgical repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants

Abstract

BackgroundAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital malformation in infants presenting with angina, dyspnea, and excessive perspiration invoked by crying and feeding. The aim of this study was to evaluate the factors affecting morbidity and the use of extracorporeal membrane oxygenation (ECMO) and to assess the quality of life post-repair.MethodsIn this retrospective study, information about five infants who underwent ALCAPA repair was collected from the databases of two tertiary referral cardiac centers (King Abdulaziz University Hospital and King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia) from 2011 to 2018. The patients were diagnosed using echocardiography, and data including mitral insufficiency and ejection fraction were assessed preoperatively and postoperatively. Quality of life was assessed using a questionnaire-based interview.ResultsThe median (range) age at the time of repair was 95 (34–144) days, and the median weight was 4.9 (3–5.7) kg. Two patients underwent the Takeuchi procedure, and three patients underwent left coronary artery reimplantation and translocation to the aorta. The median preoperative ejection fraction was 25 (12.5–45)%, and at the last follow-up, the median EF of the three survivors was 59 (50–70)%. There was no significant change in mitral insufficiency grade postoperatively. Two patients had ECMO support and died postoperatively. The infants who died were younger (< 75 days) and had lower weight (< 4.5 kg) at the time of intervention. Patients who survived showed good quality of life were asymptomatic and had heart function within the normal range.ConclusionEchocardiography and CT angiogram are effective tools for ALCAPA diagnosis in pediatric patients. Low weight at time or repair was associated with increased morbidity and ECMO did not increase survival.