静脉导管缺失的产前超声诊断及妊娠结局分析

Prenatal ultrasonographic diagnosis and pregnancy outcomes of absent ductus venosus

目的研究静脉导管缺失(absent ductus venosus,ADV)胎儿的产前超声图像特征及其与妊娠结局的关系。方法回顾性分析2017年1月—2020年2月期间在复旦大学附属妇产科医院超声科行高危超声检查诊断为静脉导管缺失的21例胎儿的超声图像和妊娠结局。根据Achiron等的新分类方法,按脐静脉-门静脉系统中分流部位进行产前超声分型:Ⅰ型为脐静脉-体静脉分流(umbilical-systemic shunts,USS);Ⅱ型为“静脉导管”-体静脉分流(ductus venosus-systemic shunts,DVSS);Ⅲ型为门静脉-体静脉分流(portal-systemic shunts,PSS);Ⅲ型分为Ⅲa型肝内门-体静脉分流(intrahepatic portal-systemic shunts,IHPSS)和Ⅲb型肝外门-体静脉分流(extrahepatic portalsystemic shunts,EHPSS)。通过查阅病史、收集影像学报告和电话随访妊娠结局至产后42天(活产、产后超声、肝功能检查或常规体检结果)的资料。结果21例ADV胎儿分为3种类型:Ⅰ型(USS)10例(47.6%),Ⅱ型(DVSS)7例(33.3%),Ⅲa型(IHPSS)4例(19.0%)。USS型病例中有6例(60%)合并心脏增大;IHPSS型病例中2例(50%)肝内门静脉-肝静脉瘘胎儿心脏增大;而DVSS型病例未合并心脏增大(P=0.012)。合并心内外畸形共16例(76.2%),在上述3种类型中分别为9例(90%)、4例(57.1%)和3例(75%)(P=0.288)。合并胎儿生长受限共3例,在上述3种类型中所占比例分别为10%、28.6%和0(P=0.309)。妊娠结局:5例失访;9例引产,均合并严重胎儿畸形;7例活产,USS型、DVSS型和IHPSS型活产数分别为1例(10%)、4例(57.1%)和2例(50%)(P=0.077)。结论本研究中ADV胎儿3种类型具有显著的超声特征。USS型胎儿合并心脏增大及心内外畸形率高,妊娠结局差;ADV胎儿的妊娠结局与分型及是否合并心脏增大和畸形有关。

Objective To study the prenatal ultrasonographic findings of absent ductus venosus(ADV)and its relationship with pregnancy outcomes.Methods Sonograms and clinical data of 21 ADV cases who visited ultrasound diagnosis department of Obstetrics and Gynecology,Fudan University from Jan 2017 to Feb 2020 were retrospectively reviewed.The cases of ADV were analyzed according to the new classification of Achiron,et al’s:TypeⅠwas umbilical-systemic shunt(USS);TypeⅡwas ductus venosus-systemic shunt(DVSS);TypeⅢwas divided into TypeⅢa,intrahepatic portal-systemic shunt(IHPSS)and TypeⅢb,extrahepatic portal systemic shunt(EHPSS).Fetomaternal clinical characteristics and outcomes(postnatal follow-ups to 42 days)were investigated by means of medical files,imaging documentation and telephone interviews(live birth,postpartum ultrasound,liver function,or routine physical examination results)with the mothers.Results A total of 21 cases were identified in 3 types:10(47.6%)with TypeⅠ,7(33.3%)with TypeⅡand 4(19.0%)with TypeⅢa.There were 6 cases(60%)combined with cardiomegaly in typeⅠ,2 cases(50%)combined with cardiomegaly in TypeⅢa,and there was no case combined with cardiomegaly in TypeⅡ(P=0.012).A total of 16 cases(76.2%)were combined with intracardiac and extracardiac malformations,i.e.,9 cases(90%),4 cases(57.1%)and 3 cases(75%)in the three types,respectively(P=0.288).There were 3 cases with fetal growth restriction,accounting for 10%,28.6%and 0 in the three types,respectively(P=0.309).Pregnancy outcomes:5 cases were lost to follow-up;9 cases with severe malformations were terminated of pregnancy;the remaining 7 cases were born lively,1 case(10%),4 cases(57.1%)and 2 cases(50%)in the three types,respectively(P=0.077).Conclusion In this study,there were significant ultrasonographic findings in three types of ADV.The incidences of cardiomegaly and intracardiac and extracardiac malformations were higher in USS type cases with poor outcomes.Postnatal outcome was associated with the types of ADV,the presence and severity of cardiomegaly,and associated malformations.