先天性左冠状动脉主干闭锁3例超声心动图特征及文献复习

目的 通过研究先天性左冠状动脉主干闭锁（LMCAA）的超声心动图表现,提高对LMCAA诊断的准确性.方法 回顾性分析经冠状动脉造影证实的3例LMCAA患儿的超声心动图检查结果,并复习相关文献,总结LMCAA超声心动图特征.结果 LMCAA特异性超声心动图特征：①主动脉左冠状动脉窦内无左冠状动脉主干开口,左冠状动脉主干近心端闭锁呈盲端,远心端内径细窄,发育不良;②右冠状动脉内径增宽;③多切面未显示左冠状动脉与肺动脉确切连接的证据.LMCAA非特异性超声心动图特征：①左心室明显扩大,左室收缩功能可正常或减低;②二尖瓣腱索、乳头肌回声显著增强,可伴有二尖瓣脱垂.彩色多普勒超声特征：①收缩期二尖瓣口可见中至大量反流信号;②左、右冠状动脉之间形成细小侧支循环; ③左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;④发育不良的左冠状动脉虽然在肺动脉周围分布,但彩色多普勒超声不能显示其与肺动脉连接的确切逆灌血流信号.结论 LMCAA有特异性的超声心动图特征,提高对LMCAA的全面认识是诊断本病的关键.

The echocardiographic features of 3 cases with congenital atresia of the left main coronary artery and literature review

Objective To improve the diagnostic accuracy of congenital atresia of the left main coronary artery by analyzing echocardiography results. Methods Three cases with congenital atresia of the left main coronary artery were diagnosed by coronary arteriongraphy. The echocardiography characteristics were summarized by reviewing the related literatures. Results The specific echocardiography characteristics include： （1）There was no left main coronary artery ostium in the left coronary artery sinus. The proximal part of left main coronary artery was atretic and the far part was narrow and dysplasia. （2） The diameter of right main coronary arteries was widened. （3）There was no anomalous origin of the left coronary artery from the pulmonary artery. The non- specific echocardiography characteristics include： （4）The left ventricular chamber was enlarged significantly and the left ventricular systolic function was normal or reduced. （5）The echo of mitral chordae tendineae and papillary muscle was enhanced and associated with mitral valve prolapsed. The color Doppler echocardiography characteristics include： （1）Color Doppler showed moderate or severe mitral valve regurgitation. （2） The small and thin collateral vessels were established between the left coronary artery and right coronary artery. （3）The flow of left coronary descending and circumflex branch was inversive. @Although the dysplasia left coronary artery was distributed around the pulmonary artery, color Doppler did not show the exact inversive flow where left coronary artery connected with pulmonary artery. Conclusions Congenital atresia of the left main coronary artery has specific echocardiography characteristics. The comprehensive understanding for this anormaly is the keypoint to make acurate diagnosis.