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目的:探讨冠状动脉异常起源于肺动脉畸形的临床特点，总结其诊断、治疗及预后经验。方法:回顾性分析2009年1月至2018年9月在北京儿童医院心脏外科就诊的所有单纯的冠状动脉异常起源于肺动脉的患儿临床资料。共37例患儿纳入研究，其中女15例，男22例；所有患儿均经超声心动图及心脏增强CT检查确诊，诊断时年龄为(15.5±20.5)个月，范围为1个月14 d至6岁7个月。37例患儿中，有10例放弃治疗离院，余27例均接受了积极治疗。27例接受治疗的患儿中，19例冠状动脉侧支循环为成人型，8例为婴儿型；1例术前突发呼吸循环衰竭而死亡，26例接受了手术治疗。26例手术患儿中，仅1例二尖瓣无反流，余25例均存在反流，且其中1例同时合并左心室室壁瘤。对所有离院或出院患儿进行随访，分析影响患儿术后早期死亡的危险因素。结果:26例手术患儿中，术后在院死亡3例，其余23例痊愈出院后存活良好。10例放弃治疗离院患儿中，除1例因左、右冠状动脉之间存在丰富侧支血管存活至今，其余9例均于离院后1年内死亡。单因素分析结果显示，术前射血分数、术前左心室后壁运动幅度、术中主动脉阻断时间、侧支循环为婴儿型是导致患儿术后早期死亡的危险因素(n P<0.05)。多因素Logistic回归分析显示，术前射血分数、侧支循环为婴儿型、合并室壁瘤是导致患儿术后早期死亡的独立危险因素(n P<0.05)。n 结论:超声心动图和心脏增强CT检查是诊断冠状动脉异常起源于肺动脉畸形较为良好的影像学方法，手术是该疾病唯一的治疗手段。诊断明确应及早行手术治疗，手术重建双冠状动脉系统后可取得较为理想的效果。“,”Objective:To explore the clinical characteristics of anomalous left coronary artery originating from pulmonary artery (ALCAPA) and to summarize its diagnosis, treatment and prognosis.Methods:From January 2009 to September 2018, retrospective analysis of clinical data was performed for 37 children of simple ALCAPA. There were 22 boys and 15 girls. They were diagnosed by echocardiography and enhanced cardiac computed tomography (CT). The diagnostic age was (15.5±20.5) months (1 month, 14 days to 6 years, 7 months). Except for 10 children giving up treatment and leaving hospital, the remainder were actively treated (n=27). The clinical types were adult (n=19) and infant (n=8). One child died preoperatively of sudden respiratory and circulatory failure. Among 26 survivors, only 1 was free from mitral valve regurgitation while another had concurrent left ventricular aneurysm. Follow-ups were conducted after discharge. And the risk factors of early postoperative mortality were analyzed.Results:Among 26 operated children, except for 3 postoperative deaths, the remainder survived, recovered and were discharged. One child survived due to abundant collateral vessels between left and right coronary arteries. And 9 deaths occurred within 1 year after discharge. Univariate analysis revealed that preoperative ejection fraction, preoperative left ventricular posterior wall motion amplitude, intraoperative aortic block time and infantile collateral circulation were risk factors for early postoperative death in children (n P<0.05). Multivariate logistic regression analysis indicated that preoperative ejection fraction, infantile collateral circulation and combined ventricular aneurysm were independent risk factors for early postoperative death in children (n P<0.05).n Conclusions:Echocardiography and enhanced cardiac CT are excellent imaging tools for diagnosing ALCAPA. Surgery is a sole treatment. Once a definite diagnosis is made, prompt surgery should be performed as early as possible. And double coronary artery system may be obtained with a better efficacy.