探讨左心室射血分数（LVEF）减低的左冠状动脉异常起源于肺动脉（ALCAPA）的超声心动图特点及手术效果。

选取2008年10月至2018年9月首都医科大学附属北京儿童医院ALCAPA患者28例，根据LVEF分为LVEF减低组（LVEF＜50%）19例和LVEF正常组（LVEF≥50%）9例，比较2组患者超声心动图表现，应用两独立样本t检验比较2组患者间超声参数的差异。手术患者（17例）分别于术后1 d、1个月、6个月、1年进行超声心动图随访，应用配对t检验与术前超声参数进行对比。

LVEF减低组患者LVEF为30%~48%，平均年龄5.4个月；LVEF正常组LVEF为60%~73%，平均年龄2.5岁。LVEF减低组与LVEF正常组相比，左心室舒张末期内径大［（131.95±24.24）mm/m² vs（85.67±20.26）mm/m²］，右冠状动脉内径小［（3.06±0.51）mm vs（4.01±0.62）mm］，差异具有统计学意义（t=-4.465、4.075，P均＜0.001），易形成室壁瘤。LVEF减低组中10例行手术治疗，7例超声心动图随访至术后1年；与术前相比，术后1 d开始左心室舒张末期内径逐渐回缩［（109.98±16.06）mm/m²、（99.49±12.02）mm/m²、（89.48±10.90）mm/m²、（80.03±8.09）mm/m² vs（123.26±12.40）mm/m²］，差异具有统计学意义（t=-2.668、-7.519、-5.413、-6.526，P=0.004、0.001、0.012、0.007），LVEF术后1个月开始恢复，术后6个月达正常水平［（45.50±9.57）%、（66.25±10.34）%、（67.25±4.50）% vs（38.83±7.05）%］，差异具有统计学意义（t=3.162、3.683、5.869，P=0.025、0.014、0.002）。

LVEF减低的ALCAPA患儿年龄小、心脏病变重，超声心动图可以准确作出诊断，但需要与心内膜弹力纤维增生症、扩张型心肌病相鉴别。ALCAPA手术治疗远期效果较好，超声心动图在术后随访中有重要作用。

To explore the characteristics and surgical efficacy of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) with reduced left ventricular ejection fraction (LVEF) by using echocardiography.

A total of 28 patients with ALCAPA treated at Beijing Children's Hospital were enrolled in this study from October 2008 to September 2018. According to the measurement of LVEF, the patients were divided into two groups: reduced LVEF group (LVEF＜50%, n=19) and normal LVEF group (LVEF≥50%, n=9). The echocardiographic features of the two groups were compared by independent sample t-test. Post-operative patients (n=17) of the two groups were followed by echocardiography, and the data at 1 day, 1 month, 6 months and 1 year after operation were compared with pre-operative data by compared t-test.

There were 19 patients in the reduced LVEF group (LVEF: 30% to 48%) with an average age of 5.4 months, and 9 patients in the normal LVEF group (LVEF: 60% to 73%) with an average age of 2.5 years. Compared with the normal LVEF group, the left ventricular end-diastolic dimension was larger in the reduced LVEF group [(131.95±24.24) mm/m² vs (85.67±20.26) mm/m²; t=-4.465, P＜0.001], and the inner diameter of the right coronary artery was smaller [(3.06±0.51) mm vs (4.01±0.62) mm; t=4.075, P＜0.001]. Ventricular aneurysms were prone to occur in the patients of the reduced LVEF group. In the reduced LVEF group, ten patients underwent surgical treatment, and seven were followed by echocardiography from 1 day to 1 year after surgery; the left ventricular end-diastolic dimension gradually retracted from 1 day to 1 year after surgery [(109.98±16.06) mm/m², (99.49±12.02) mm/m², (89.48±10.90) mm/m², and (80.03±8.09)mm/m² vs (123.26±12.40) mm/m²; t=-2.668, -7.519, -5.413, and -6.526; P=0.004, 0.001, 0.012, and 0.007, respectively]. The LVEF started to recover 1 month after operation, and reached the normal level at 6 months after operation [(45.50±9.57)%, (66.25±10.34)%, and (67.25±4.50)% vs (38.83±7.05)%; t=3.162, 3.683, and 5.869; P=0.025, 0.014, and 0.002, respectively).

Children with ALCAPA with reduced LVEF are younger and have a more severe heart disease. Echocardiography can make an accurate diagnosis, but ALCAPA needs to be differentiated from endocardial fibroelastosis and dilated cardiomyopathy. ALCAPA surgery has a better long-term effect, and echocardiography plays an important role in postoperative follow-up.