摘要目的 探讨左冠状动脉异常起源于肺动脉(ALCAPA)的临床表现、诊治及预后.方法 回顾性总结分析上海交通大学医学院附属上海儿童医学中心2010年3月至2017年8月收治的91例ALCAPA患儿的病例资料,按就诊时左心室射血分数(LVEF)分为心功能不全组54例和心功能正常组37例,对比分析两组患儿的临床特征(发病年龄、临床表现等)与辅助检查(心电图、心脏超声等),组间比较采用t检验或χ2检验,对预后影响因素进行有序Logistic回归、Pearson相关系数分析.结果(1)心功能不全组患儿确诊年龄为(10.0±2.6)月龄,易被误诊为扩张型心肌病(20/54);心功能正常组患儿确诊年龄为(40.0±7.8)月龄,易误诊为二尖瓣病变(4/37).按病理生理循环划分,心功能不全组74%(40/54)为婴儿型,心功能正常组78%(29/37)为成人型.(2)术前心电图提示心功能不全组I导联深Q波例数多于心功能正常组(28比11例,χ2=4.388,P=0.036).(3)心功能不全组患儿死亡20例,其中12例因术后心泵功能衰竭死亡,8例患儿因预后差等原因未行手术治疗后死亡;心功能正常组无死亡病例.(4)术前LVEF为影响预后的唯一因素(F=16.872,P=0.005),心功能不全组患儿术前LVEF为(37±11)%,术后为(45±14)%,差异有统计学意义(t=3.614,P=0.001).心功能不全组6例患儿术后心功能仍低下;心功能正常组患儿随访心功能正常.结论 术前LVEF为影响疾病预后的唯一因素,术前心功能不全的婴儿型ALCAPA患儿术后需进行长期随访治疗.

abstractsObjective To explore the clinical manifestations, diagnosis, treatment and prognosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Methods A retrospective study identified 91 patients diagnosed with ALCAPA at Shanghai Children′s Medical Center from March 2010 to August 2017. According to the left ventricular ejection fraction (LVEF) at the time of consultation, patients were divided into the cardiac insufficiency group (n=54) and the normal cardiac function group (n=37). Clinical features (age of onset, clinical performance, etc) and auxiliary examinations (electrocardiogram, echocardiography, etc) between the two groups were compared using a t‐test and a Chi‐square test. Prognostic factors were analyzed by an ordered logistic regression and a Pearson correlation coefficient. Results (1) The age of diagnosis of patients in the cardiac insufficiency group who were usually misdiagnosed as cardiomyopathy was (10.0±2.6) months (20/54), whereas the age of diagnosis of patients in the normal cardiac function group who were usually misdiagnosed as valvular diseases was (40.0 ± 7.8) months (4/37). According to the pathophysiological mechanism, forty of the 54 (74%) patients in the cardiac insufficiency group were infantile type, and 78% patients (29/37) in the normal cardiac function group were adult type. (2) Preoperative electrocardiogram showed the deep Q wave in lead I occurred more frequently in the cardiac insufficiency group than in the normal cardiac function group (28/54 vs. 11/37, χ2=4.388, P=0.036). (3) Twenty patients died in the cardiac insufficiency group including 12 patients who died from postoperative cardiac pump failure and 8 children who did not undergo surgery due to poor prognosis and died from other reasons. There was no death in the normal cardiac function group. (4) Preoperative LVEF was the unique risk factor affecting prognosis (F=16.872, P=0.005). The preoperative LVEF was significantly lower than the postoperative LVEF ((37±11)% vs. (45±14)%, t=3.614, P=0.001) in the cardiac insufficiency group. During the follow‐up period, 6 patients in the cardiac insufficiency group still presented with postoperative cardiac dysfunction, and the patients in the normal cardiac function group still had normal cardiac function. Conclusions Preoperative LVEF was the unique risk factor affecting prognosis of ALCAPA. Patients with infantile type ALCAPA and preoperative cardiac insufficiency should receive long‐term follow‐up treatment.