成人型与婴儿型左冠状动脉异常起源于肺动脉的超声诊断

The echocardiography diagnosis of infantile and adult types of anomalous origin of the left coronary artery from the pulmonary artery

目的总结不同类型左冠状动脉异常起源于肺动脉(ALCAPA)的超声心动图表现,提高对本病的诊断及鉴别诊断能力。方法回顾性分析首都医科大学附属北京安贞医院儿童心血管病中心收治并经手术证实的24例ALCAPA患儿的超声心动图结果,根据侧支循环的发育情况分为婴儿型和成人型,并总结婴儿型和成人型ALCAPA的超声心动图特征。结果婴儿型和成人型ALCAPA相同的超声心动图特征:①左冠状动脉窦内无左冠状动脉主干开口,其主干直接与肺动脉连接;②左心室高度扩张,室间隔和左室前壁节段性运动障碍,左室收缩功能减低;③左心室广泛纤维化,以心内膜下区域最为显著;④二尖瓣腱索、乳头肌纤维化,回声显著增强;⑤右冠状动脉起源位置正常,内径增宽;⑥左冠状动脉前降支和回旋支血流为逆向灌注(向心性);⑦收缩期二尖瓣口可见少至大量反流信号。婴儿型和成人型ALCAPA不同的超声心动图表现:彩色多普勒显示成人型侧支循环血流丰富,婴儿型侧支循环少。结论婴儿型和成人型ALCAPA具有其特异性的超声心动图特征,结合其临床及心电图等表现,可以对本病做出正确诊断。

Objective To improve the diagnosis and differential diagnosis ability for different type of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA)by summarizing patient's echocardiography features and in combination with its clinical and electrocardiogram performances. Methods The echocardiography results of twenty-four cases with confirmed diagnosis of ALCAPA by surgery were retrospectively reviewed. According to the development of coronary artery collateral circulation,patients were divided into infantile type and adult type. The different and the common echocardiography features were summarized in two types of ALCAPA. Results The same echocardiography features of infantile and adult types:①There was no left main coronary artery ostium in left coronary cusp,in stead,the left coronary artery( LCA)was originated from pulmonary artery. ②Left ventricle chamber was enlarged obviously. The segments movement of interventricular septum and anterior wall of left ventricle was disorderd. The systolic function of left ventricle was reduced. ③The fibrosis of left ventricle was extensive and the area of endocardium was significant. ④The echo of mitral chordae tendineae and papillary muscle was enhanced. ⑤The diameter of right coronary artery( RPA)was significantly dilated. ⑥The flow of left coronary descending and circumflex branch was inversed.⑦The mitral regurgitation was mild or severe. The following features differed in infantile and adult types. The significant collateral vessels were established between the RCA and LCA in adult type,however it was not the case for infantile type. The collateral vessels were less in infantile type. Conclusion The different types of ALCAPA have some specific echocardiography features. Combining patient 's echocardiography features with clinical and electrocardiogram performances,the accurate diagnosis can be achieved.