单纯性完全型肺静脉异位引流产前8例超声心动图诊断

摘要 目的 探讨胎儿单纯性完全型肺静脉异位引流（TAPVC）的产前超声心动图特点，提高对本病的产前诊断准确率。方法 回顾性分析2011年5月至2014年2月经新生儿超声心动图、手术或尸解证实的8例单纯性TAPVC的胎儿期超声心动图检查结果，总结超声心动图特征。结果 8例单纯性TAPVC胎儿中，心下型2例，心内型1例，心上型5例，5例存在垂直静脉或共同肺静脉腔与垂直静脉连接处梗阻；8例胎儿中，引产1例（心下型），余7例均于出生后行手术治疗，其中1例术后死亡（心下型），6例均恢复良好。TAPVC胎儿超声心动图特征为：①正常的左心房形态消失，呈圆形或椭圆形，左心房光滑并多变小，在孕后期较为明显；②降主动脉与左心房间距离明显增大，多数在左心房后方可见一异常的腔隙（即共同肺静脉腔），可显示左、右侧上升（心上型）或下降（心下型）的垂直静脉；③妊娠早期左、右心系统比值早期多正常，但妊娠中、晚期（孕26周后）可出现右心系统轻度扩大；④引流入冠状静脉窦时（心内型），冠状静脉窦可有扩张；引流入上腔静脉时（心上型），上腔静脉扩张；引流入肝内血管时（心下型），肝内血管可有不同程度的扩张；⑤彩色多普勒可显示引流途径及是否合并垂直静脉梗阻。结论 在孕早期左、右心系统比值正常时，TAPVC易被漏诊及误诊，应注意多角度、多切面扫查，孕晚期超声心动图检查可减少漏诊。

Prenatal diagnosis of isolated total anomalous pulmonary venous connection by fetal echocardiography

Abstract Objective To improve the prenatal diagnosis accuracy of isolated total anomalous pulmonary venous connection (TAPVC) by analyzing and accumulating fetal echocardiography features accurately diagnosed by fetal echocardiography. Methods Fetal echocardiographic signs and accumulated fetal echocardiography features were retrospectively analyzed in 8 cases with prenatal diagnosis of isolated TAPVC which was confirmed by neonatal echocardiography, surgery or autopsy from May 2011 to February 2014. Results Diagnosis of TAPVC was made in 8 fetuses, including 5 with supracardiac connection, 2 with infracardiac connection and 1 with cardiac connection. Only 2 fetuses were diagnosed TAPVC in other hospitals before, including 1 fetus diagnosed as abnormal vessel in liver and infracardiac TAPVC, 1 fetus diagnosed as infracardiac TAPVC combined with descending vertical vein obstruction. Other 6 fetuses were all misdiagnosed, including 2 with hypoplastic left heart syndrome and coarctation of aorta, 1 with dilated coronary sinus and left superior vena cava, 1 with abnormal vessal nearby superior vena cava combined with stenosis, 1 with left superior vena cava and 1 with small diameter of left heart. Stenosis along the vertical vein pathway or the connection of the vertical vein to pulmonary venous confluence was identified in 5 fetuses. In 1 terminated of pregnancy case with infracardiac connection, autopsy confirmed the prenatal diagnosis. Other 7 fetuses were born and performed surgery after birth, 1 case with infracardiac connection died in 1 week after surgery and other cases recovered well. The echocardiography characters of fetuses diagnosed with TAPVC：① The normal shape of left atrium disappeared and left atrium became round or oral and narrow especially in the latter phase of pregnancy. ② The distance was increased between left atrium and the descending aorta (Dao) and an abnormal pulmonary venous confluence presented posterior to the left atrium in most cases, and the ascending vertical vein or descending vertical vein could be seen. ③ The ventricular proportion was normal at the earliest gestation, but the right heart was dilated after 26 weeks. ④ A dilated coronary sinus could be seen in fetuses with cardiac TAPVC and a dilated superior vena cava in the fetus with supracardiac TAPVC and the dilated hepatic vessel in infracardiac TAPVC fetuses as well. ⑤ Color Doppler imaging could show the draining trace and whether existed vertical vein obstruction. Conclusion The isolated TAPVC is easy to be misdiagnosed when the ventricular proportion is normal at the earliest gestation. More attention should be paid on more angle and more exam views when performing fetal echocardiography. The fetal echocardiography can reduce misdiagnosis at the latter phase of pregnancy.