ObjectiveTo explore surgical methods and risk factors of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). MethodsClinical data of 28 ALCAPA patients who underwent surgical repair from October 1993 to September 2013 in Beijing Anzhen Hospital were retrospectively reviewed. There were 8 male and 20 female patients with their age of 0.6-l6.8 (4.3±0.7)years including 10 patients less than 1 years old. Surgical procedures included simple ligation of left coronary artery, intrapulmonary tunnel procedure (Takeuchi)and direct coronary reimplantation of the anomalous artery. Postoperative death, complication and cardiac function were observed. ResultsAmong the 28 patients, 1 patient received simple ligation of left coronary artery, and 7 patients received intrapulmonary tunnel procedure (Takeuchi), among whom 2 patients died postoperatively. Twenty patients received direct implantation of the anomalous artery into the ascending aorta, and 3 patients died postoperatively. Five patients who died postoperatively were 10.20±3.27 months old, including 3 patients with moderate mitral regurgitation (MR)and 2 patients with mild MR preoperatively. Preoperative heart function of the patients who died postoperatively was significantly reduced. Preoperative left ventricular ejection fraction of the patients who died postoperatively was significantly lower than that of the patients who survived (36.6%±8.5% vs. 60.9%±10.7%, P=0.000). Low cardiac output syndrome was the reason for all postoperative death. All survival patients were followed up from 1 month to 18 years. One patient who underwent intra-pulmonary tunnel procedure (Takeuchi)received pulmonary artery balloon dilatation for pulmonary supravalvular stenosis 15 years after discharge. None of the other patients received a secondary operation. During follow-up, left ventricular function was improved. Growth and development of all the patients was normal. MR did not significantly aggravate in all the patients. ConclusionPatients with younger age and worse left ventricular function have greater surgical risks of ALCAPA.