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.