Diagnosis and surgery of anomalous origin of the left coronary artery from the pulmonary artery in children

Abstract

To explore the clinical features, diagnosis and management of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in children. The clinical manifestations, examinations, diagnostic modalities and treatments of children identified as ALCAPA at Beijing Anzhen Hospital from September 1993 to September 2011 were retrospectively reviewed. A total of 27 children were recruited. There were 16 males and 11 females with an age range of 1 month to 13 years. Among them, 19 patients had an onset age of ≤ 1 years. The initial symptoms were acute heart failure (n = 15), pneumonia (n = 7) and cardiac dilation with murmur (n = 5). The pre-admission misdiagnoses included endocardial fibroelastosis (EFE) (n = 13), dilated cardiomyopathy (DCM) (n = 4) and mitral severe regurgitation (n = 2). The definite diagnosis was established on the basis of electrocardiography (ECG), echocardiography and 64-slice computed tomography or angiography. Twenty-six cases underwent immediate surgery after an accurate diagnosis, including left coronary artery (LCA) ligation (n = 1), LCA ligation plus coronary artery bypass grafting (n = 1), Takeuchi operation (n = 7) and LCA reimplantation (n = 17). Six died postoperatively. And 20 cases were routinely followed up with symptoms, signs, ECG and echocardiography for 3-192 months. The outpatient visits were at Months 3, 6 and 12 post-operation and then annual check-ups by returning to hospital, telephone, letters and other forms. The cohort had no overt symptoms and left ventricular ejection fraction returned to normal range. No complications occurred after LCA reimplantation. As a rare congenital heart anomaly, ALCAPA may be definitely diagnosed with clinical features and serial diagnostic methods. It can be treated with several types of cardiac surgery. Coronary reimplantation is the technique of choice for surgical correction of ALCAPA due to its excellent prognosis.