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目的:总结评估单中心15年对于左冠状动脉异常起源于肺动脉(anomalous origin of the left coronary artery from the pulmonary artery，ALCAPA)的外科治疗经验及预后评估。方法:回顾性分析2003年1月至2018年9月上海儿童医学中心心胸外科收治的126例ALCAPA患儿的临床资料，其中男59例，女67例，年龄为(17.8±12.6)个月，体重为(8.1±3.4)kg。所有患儿均接受ALCAPA纠治术，手术方式包括65例采用“凸窗”技术，51例利用自体组织重建冠状动脉管道(22例肺动脉组织管道，15例心包管道以及14例双活瓣延长管道)，10例行“Takeuchi”技术；所有患儿中有72例合并中度以上二尖瓣反流，同时接受了二尖瓣整形手术。结果:21例患儿因急性左心衰竭于术后72 h内行机械辅助循环，辅助3～7 d，均顺利撤机。术后早期11例患儿死亡，其中4例为机械辅助撤离后死亡，死亡原因主要为左心衰竭、多器官衰竭和脑出血。3例中远期死亡发生在术后18个月内，死亡原因为心脏休克和心力衰竭。生存患儿出院后规律随访，随访时间为(65.0±36.2)个月，心脏彩色多普勒超声检查结果显示：所有患儿新建的冠状动脉均血流通畅，灌注良好；左心室射血分数(left ventricular ejection fraction，LVEF)由术前的(46.2±15.0)%提高至(60.2±11.7)%(n P0.05)。5例患儿在随访期间行二次手术，再手术率为4.5%。n 结论:根据冠状动脉的解剖特点采用个体化手术方式治疗ALCAPA的手术效果满意，中远期随访的患儿心功能明显好转，预后良好；二尖瓣整形术能有效改善瓣膜反流，且不增加ALCAPA患儿手术风险；机械辅助循环是重症ALCAPA患儿术后早期安全有效的过渡手段。“,”Objective:To assess the midterm surgical outcomes for children with an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) for 15 years at a single center.Methods:In this retrospective study, 126 surgical ALCAPA children were recruited from January 2003 to September 2018. There were 59 boys and 67 girls with a mean age of (17.8±12.6) months and a mean body weight of (8.1±3.4) kg. The surgical approaches included “Bay Window” technique (n=65), autologous tissue conduit reconstruction (n=51, 22 via a pulmonary conduit, 15 via a pericardial conduit and 14 via a double flaps conduit), Takeuchi technique (n=10) and mitral annuloplasty or valvuloplasty for moderate/severe mitral regurgitation (n=72, MR).Results:Twenty-one children with acute heart failure received postoperative mechanical circulatory supports within 72 hours for 3-7 days. There were 11 in-hospital deaths with a mortality rate of 8.7% including 4 children weaning from mechanical circulatory support. The early postoperative causes of death were acute myocardial failure, multiple organ failure and pyemia. Three delayed postoperative deaths occurred within 18 months because of cardiac insufficiency and sudden death. During a mean follow-up period of (65.0±36.2) months, echocardiography indicated that all survivors had fluent coronary flow; LVEF significantly improved from preoperative (46.2±15.0)% to (60.2±11.7)% during follow-ups (n P0.05). Five patients (4.5%) were re-operated during follow-ups.n Conclusions:Individualized surgical approaches based upon different coronary anatomies and preoperative cardiac functions have an acceptable survival rate and favorable midterm outcomes. MV does not increase the risk of surgery; postoperative mechanical circulatory assist device is an effective transitional support for severe ALCAPA patients.