Abstract: Objective:To analyze the echocardiographic characteristics of anomalous left coronary artery from the pulmonary artery (ALCAPA) with normal left ventricular systolic function. Methods:The echocardiographic characteristics and clinical data of 13 ALCAPA patients with left ventricular ejection fraction (LVEF) ≥55% were retrospectively analyzed. All of the included 13 patients were admitted to Guangzhou Women and Children's Medical Center from January 2010 to December 2019. Results:Right coronary artery dilation was revealed in all of the 13 patients. Other echocardiographic markers included left coronary artery flow reversal (12 cases), visualization of coronary artery origin from the pulmonary trunk (9 cases), abnormal doppler signal in pulmonary trunk (7 cases), collateral vessels in myocardium (8 cases), left ventricular enlargement (8 cases), mitral regurgitation (7 cases) and endocardial fibroelastosis (5 cases). Patients ＜1 year were more likely to be lack of collateral coronary artery circulation，and showed normal or mild enlarged left ventricle with normal mitral function, while patients ≥1 year always showed significant collateral formation, dilated left ventricle and mitral dysfunction. In this study, four misdiagnosed patients showed that the left coronary artery originated from the right-rear side of pulmonary artery. Conclusion：The echocardiography can diagnose ALCAPA with normal left ventricular systolic function accurately. The rate of misdiagnosis and missed diagnosis will be reduced if thorough echocardiographic interrogation of coronary artery origins and flow is carried out.