先天性左冠状动脉主干闭锁的超声心动图诊断

Diagnosis of Congenital Left Main Coronary Artery Atresia with Echocardiography

目的探讨超声心动图对先天性左冠状动脉主干闭锁(LMCAA)的诊断价值。方法回顾性分析9例LMCAA患儿的超声心动图特点,与冠状动脉CT或造影结果以及手术情况进行对比,并分析其短期随访结果。结果本组9例患儿,超声检查明确诊断7例,超声诊断LMCAA最重要的征象包括:(1)左心房室内径增大;(2)左冠状动脉近端闭锁、发育细小或无法显示;(3)二尖瓣腱索回声增强呈缺血性改变,二尖瓣中量及以上反流。其余征象还包括:(1)节段性或弥漫性左心室壁运动异常,左心室收缩功能减低;(2)左心室壁或室间隔内探及侧支血流;(3)左冠状动脉内探及逆向血流。其中二尖瓣的缺血性改变对此病诊断特异性高。短期随访发现年龄偏大的患儿术后仍存在明显的二尖瓣反流。结论超声心动图对于LMCAA具有重要的诊断价值,早期诊断和治疗或可改善其预后。

Objective To explore the value of the echocardiography in the diagnosis of congenital left main coronary artery atresia(LMCAA).Methods The echocardiographic characteristics of 9 children diagnosed with LMCAA were retrospectively analyzed,and compared with the results of coronary angiography or computed tomography(CT)and surgical findings.In addition,their short-term prognosis were followed up.Results LMCAA is an extremely rare and subtle deformity.In this group of 9 patients,7 cases were definitely diagnosed with LMCAA by echocardiography.The most important echocardiographic characteristics are:enlarged left heart chambers,the proximal coronary artery is small or unclear,moderate to severe mitral valve regurgitation with the ischemic chordae tendineae.There are some other characteristics including segmental or diffuse left ventricular wall motion abnormalities with decreased left ventricular systolic function;collateral blood flow in left ventricular wall or ventricular septum;reverse blood flow in left coronary artery.Of which,ischemic manifestations of the mitral valve has high diagnostic specificity.Echocardiography demonstrated that mitral regurgitation of older children did not improve significantly postoperatively.Conclusion Echocardiography has important diagnostic value for LMCAA,which could lay the foundation for the timely treatment of patients,or improve their prognosis.