中文摘要：

目的观察静脉导管缺如（ADV）并脐静脉肝内、肝外异常连接的产前超声心动图表现。方法回顾性分析14胎ADV胎儿的超声心动图表现,观察心脏大小、功能变化,并随访临床预后。结果 ADV的检出率为0.12%（14/11205）,14胎中,5胎脐静脉与门静脉左支、1胎与门静脉右支相连;8胎脐静脉未与门静脉相连,而与肝外静脉系统相连,5胎直接连接于右心房,1胎直接连接于下腔静脉,1胎连接于肝右静脉,1胎连接于冠状静脉窦引流入右心房。结论产前超声心动图能够明确诊断静脉导管缺如,且可以观察脐静脉肝内、肝外异常连接途径和管道内径。

英文摘要：

Objective To observe the fetal echocardiography characteristic of agenesis of the ductus venosus（ADV）with abnormal umbilical vein connection.Methods The fetal echocardiography characteristics of 14 fetuses with ADV were observed,and the size of heart,changes on heart function and prognosis were analyzed retrospectively.Results The detection rate of ADV was 0.12%（14/11 205）.In five cases of them,the umbilical vein was connected to the portal vein,as well as one cases to the right,whereas of them,the umbilical vein neither drained to the left portal nor right portal vein in the other eight cases.The umbilical vein connection to the extrahepatic venous system by tracking detection.In five of the eight cases,the connection was directly to the right atrium,one directly to the inferior vena cava,one to the right hepatic vein and the other drained into the right atrium through coronary sinus.Conclusion Fetal echocardiography can be used to diagnose the ADV accurately.Furthermore,it can be used to observe the umbilical vein abnormal connection intrahepatic or extrahepatic and measure the pipe diameter.