心下型完全性肺静脉异位连接的超声心动图诊断

目的 评价超声心动图对婴幼儿心下型完全性肺静脉异位连接的诊断价值。方法 回顾性分析2006年4月至2016年12月经超声心动图诊断的8例心下型完全性肺静脉异位连接患儿,其中男4例、女4例,年龄15~195 d,平均年龄79.8 d。超声从胸骨旁、剑突下、心尖多个切面,观察肺静脉异位引流的途径及位置,右心房、右心室的扩大程度,心房水平分流方向等。结果 8例心下型完全性肺静脉异位连接患儿中,6例行CT检查,5例行手术治疗。超声心动图检出7例,漏诊1例,诊断准确率为87.5%。本病的间接超声征象为小的畸形左心房和心房水平右向左分流,肝、门静脉扩张迂曲,肝内血流丰富;直接征象为左、右肺静脉未回流左心房,汇成共同肺静脉干,汇入垂直静脉后经右下行穿越膈肌至门静脉或肝静脉。剑突下切面可显示3条平行走行的血管,即垂直静脉、腹主动脉和下腔静脉,前二者与后者血流方向相反。8例患儿垂直静脉膈下回流部位均为门静脉,3例患儿存在降垂直静脉与门静脉或肝静脉连接处梗阻。结论 超声心动图对诊断小儿心下型完全性肺静脉异位连接具有重要价值,能为外科术前评估提供结构及血流动力学信息。采用多声窗、多切面及连续追踪法观察心下型完全性肺静脉异位连接异位引流的肺静脉行程,可对其作出准确诊断,但须与混合型肺静脉异位引流及肝内门-体静脉分流或肝动脉-静脉瘘等疾病相鉴别。

Echocardiographic diagnosis of infracardiac total anomalous pulmonary venous connection

Objective:To investigate the clinical usefulness of echocardiography in the diagnosis of infracardiac total anomalous pulmonary venous connection (ITAPVC) in neonates and infants.Methods:Retrospective analysis on 8 patients with ITAPVC was performed using echocardiography between April 2006 and December 2016.There were 4 boys and 4 girls with a mean age of 79.8 days (ranging from 15 to 195 days).A combined scanning via parasternal,subcostal and apical acoustic windows had been employed to diagnose ITAPVC and to trace the course and site of the anomalous pulmonary venous drainage,and to confirm the direction of the inter-atrial shunt and enlargement of right atrium and right ventricle.Results:Of the 8 patients who received echocardiography,ITAPVC was diagnosed in 7 patients.Misdiagnosis by echocardiography was encountered in one patient.The diagnosis by echocardiography was compatible with the operative findings in 5 patients receiving surgery and with the results of multislice computed tomography in 6 patients.The diagnostic accuracy rate of ITAPVC was 87.5％.The indirect signs obtained from echocardiogram was coexistence of a small malformed,triangle-shaped left atrium and right to left shunting at atrial level with dilatation and tortuousness of portal vein or hepatic vein and abundant blood flow in liver.The direct signs was total pulmonary veins unconnected with left atrium,whose confluence joining into vertical vein drained right-inferiorly to portal vein or hepatic vein through diaphragm.Three parallel vessels including vertical vein,abdominal aorta and inferior vena cava arranged anteriorly,left-posteriorly and right-posteriorly with the opposite flow directions of inferior vena cava and the other two were found on sub-costal view.Sites of the drainage to the infra-diaphragm veins located portal vein in 8 patients.Stenosis of site of vertical vein connecting to portal vein or hepatic veins occurred in 3 patients.Conclusion:Echocardiography has significant value in the diagnosis of pediatric ITAPVC and is capable of providing important structural and hemodynamic information for preoperative assessment of surgery.With multiple windows and multiple sections,ITAPVC could be diagnosed accurately by echocardiography.However,it is necessary to differentiate ITAPVC with intrahepatic portosystemic venous shunts or hepatic arteriovenous fistula.