Diagnosis and prognosis of anomalous origin of the left coronary artery from the pulmonary artery

Introduction: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital heart abnormality. The aim of this study was to describe a single-centre experience with surgical repair of this condition. Methods: We performed a retrospective analysis of cases from February 2004 to January 2014. Results: Ten patients presented with the diagnosis of ALCAPA. A total of seven infants and three adults underwent surgical repair in our Department of Thoracic Surgery, Rigshospitalet, Denmark. The seven infants presented with symptoms of heart failure: dyspnoea, sweating or failure to thrive; two adults were asymptomatic and one adult presented with cardiac arrest. Six infants had moderate to severe mitral valve regurgitation and five of these patients had preoperative moderate to severely reduced left ventricular function. Nine patients underwent surgical repair by reimplantation of the left coronary artery to the aorta and one underwent surgical repair ad modus Takeuchi (an aortopulmonary window). None of the patients underwent re-operation and none died. Conclusion: All ten patients survived with recovery of left ventricular function within 12 months. An early diagnosis and prompt surgical intervention is warranted in the treatment of ALCAPA.