胎儿三尖瓣下移畸形超声表现及预后分析的单中心研究

Ultrasonic manifestation and prognosis in fetuses with Ebstein anomaly:a single-center study

目的胎儿三尖瓣下移畸形(EA)与较差的结局相关,本研究目的是描述EA胎儿期超声心动图的特征,并评估胎儿预后。方法回顾性分析北京安贞医院心脏超声医学中心2011年1月~2020年12月于北京安贞医院就诊的53例EA胎儿和孕妇的临床资料,分析此部分EA胎儿的超声特征及预后。结果本单中心研究中,共筛查了53例EA胎儿,孕妇在妊娠23~39周之间。本研究中EA胎儿伴有并发症的37例(69.81%);超声心动图显示三尖瓣后叶总体下移程度较隔叶远,本组EA患儿中前叶出现病变的病例数<50%;严重三尖瓣反流EA患儿所占比例最高,为34例(64.15%);入组胎儿中终止妊娠40例(75.47%),继续保持妊娠13例(24.53%),最后仅1例胎儿出生。结论EA是胎儿期罕见的先天性心脏病,发病率低,患儿异质性大。超声心动图是协助诊断该病的一种重要工具。胎儿期EA的预后相对较差,因此了解胎儿期EA的超声心动图特征,对孕妇围产期预后管理具有重要的价值。

Objective To describe the features of echocardiogram in fetal patients with Ebstein anomaly(EA),and review their prognosis.Methods The EA fetuses and pregnant women(n=53)were chosen from Beijing Anzhen Hospital from Jan.2011 to Dec.2020,and their clinical materials were retrospectively analyzed.The features of echocardiogram and prognosis were reviewed in EA fetuses.Results In this single-center,there were totally 53 EA fetuses screened,and the gestation period was from 23 weeks to 39 weeks in these pregnant women.There were 37 EA fetuses(69.81%)with complicated diseases.The results of echocardiogram showed that the posterior lobe of tricuspid valve moved downward totally more than the septum.The number of anterior lobe lesions in these EA fetuses was less than 50%.The EA fetuses with severe tricuspid regurgitation accounted for the highest(34 cases,64.15%).Among the enrolled fetuses,pregnancy was terminated in 40 cases(75.47%),and sustained in 13 cases(24.53%).Finally there was only 1 fetus born.Conclusion EA is a rare congenital heart disease in the fetus with low incidence and large heterogeneity.Echocardiography is an important tool to help diagnose the disease.The prognosis of fetal EA is relatively poor,so understanding the echocardiographic characteristics of fetal EA is of great value for the perinatal prognosis management of pregnant women.