左室收缩功能正常的左冠状动脉异常起源于肺动脉患儿的超声诊断13例病例系列报告

Echocardiographic dianosis of anomalous left coronary artery from the pulmonary artery with normal left ventricular systolic function:A case series report of 13 patients

目的 探讨左室收缩功能正常的左冠状动脉异常起源于肺动脉(ALCAPA)患儿的超声心动图特点.方法 回顾性分析2010年1月至2019年12月广州市妇女儿童医疗中心收治并经手术证实的13例左心室射血分数(LVEF)≥55%的ALCAPA患儿的超声心动图特征和临床资料.结果 13例ALCAPA患儿均可见右冠状动脉增宽,其他超声征象包括左冠状动脉血流逆灌12例、左冠状动脉直接开口于肺动脉9例、肺动脉内逆灌血流7例、心肌侧枝血流8例、左室明显增大8例、二尖瓣功能不全7例、左室心内膜纤维化5例.<1岁ALCAPA患儿的心肌侧枝血流信号不明显,左心室大小正常或轻度增大且二尖瓣功能良好;≥1岁患儿可见丰富侧枝血管、左室明显扩大以及二尖瓣功能不全.4例误诊的ALCAPA左冠状动脉均起源于肺动脉右后方.结论 超声心动图可准确诊断左室收缩功能正常的ALCAPA,如果超声科医生在怀疑该诊断时能全面探查冠状动脉的起源和血流方向,可减少漏诊和误诊.

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.