62例左冠状动脉异常起源于肺动脉临床研究

摘要

目的 研究左冠状动脉异常起源于肺动脉(ALCAPA)的临床表现、治疗方法及预后.方法 回顾分析2011年1月至2016年9月收治的ALCAPA患儿的临床资料,将患儿分为婴儿组(<12个月)和儿童组(≥12个月)进行比较分析.观察左室舒张末期内径(LVDD)、左室射血分数(LVEF)、心肺转流术转流时间等.结果 62例ALCAPA患儿中婴儿组38例、儿童组24例;婴儿组中位ΔLVDD(实际测得LVDD-该年龄段LVDD正常上限)为13.6 mm(8.4～17.5 mm),儿童组中位ΔLVDD为8.5 mm(3.3～13.7 mm);婴儿组LVEF为(40.6±2.4)％,低于儿童组(59.0±2.9)％,差异有统计学意义(P<0.01).60例患儿完成手术治疗,婴儿组平均心肺转流术转流时间为(137.1±8.4)min,高于儿童组的(105.9±6.6)min,差异有统计学意义(P=0.010).婴儿组术后呼吸机支持中位时间6 d(3～7 d),长于儿童组2 d(1～4 d),差异有统计学意义(P<0.01).60例手术患儿的中位随访时间38个月,术后1个月内死亡6例(婴儿组5例、儿童组1例),以后无死亡病例;22例术后1周内出现肺动脉瓣上流速增快(>1.8 m/s).结论 ALCAPA易误诊为扩张型心肌病,婴儿型临床表现较儿童型重,术后1个月内死亡率高,远期预后较好.%Objective To explore the clinical manifestation, treatment and prognosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). Method The clinical data of patients with ALCAPA from January 2011 to September 2016 were retrospectively analyzed. All the patients were divided into infant group (<12 months) and child group (≥12 months). Results In the 62 patients (38 infants and 24 children) with ALCAPA, the median ΔLVDD (actually measured LVDD – the normal upper limit of LVDD in this age group) of infant group and child group was 13.6 (8.4～17.5) mm and 8.5 (3.3～13.7) mm respectively. The mean LVEF of the infant group was 40.6±2.4 ％, lower than that of the children group (59.0±2.9) ％, and the difference was statistically significant (P<0.01). Sixty children were treated with surgery. The mean cardiopulmonary bypass duration of infant group (137.1±8.4 min) was longer than that of the children group (105.9±6.6 min), and the difference was statistically significant (P=0.010). The median mechanical ventilation time after operation in infant group (6 d, 3～7 d), was longer than that in the children group (2 d, 1～4 d), and the difference was statistically significant (P<0.01). The median follow-up time of 60 patients was 38 months. Six patients died within 1 month after surgery (5 cases in the infant group and 1 in the children group), and there were no deaths more than 1 month after surgery. In 22 cases, the flow rate of pulmonary valve increased rapidly within 1 week after operation (>1.8 m/s). Conclusion ALCAPA is easily misdiagnosed as dilated cardiomyopathy. The clinical manifestations of infants were more severe than those of children. The mortality was high within 1 month after surgery, and the long-term prognosis was better.