Ebstein畸形的产前超声心动图诊断标准及预后评估

Assessment of the novel diagnostic criteria and prognosis of Ebstein anomaly by fetal echocardiography

目的评价超声心动图对胎儿Ebstein畸形(EA)的诊断价值,建立新的诊断标准,探索EA的预后评估指标。方法选取产前超声诊断为EA胎儿37例为病例组,孕周与病例组相匹配的正常胎儿37例为对照组,对比分析二者间的超声测量指标;宫内死亡、新生儿死亡及术后死亡者16例为死亡组,进行外科手术及随访者21例为存活组,比较二者间心胸比(CTR)、GOSE评分、动脉导管分流方向、肺动脉发育情况。结果与对照组比较,病例组胎儿CTR增大(0.49±0.28)vs(0.28±0.05),P<0.01;二尖瓣前瓣根部与三尖瓣隔瓣根部距离(MTD)明显增大(7.80±2.39)mm vs(3.80±1.20)mm,P<0.01;肺动脉与主动脉内径之比(PA/AO)明显减低(0.94±0.23)vs(1.12±0.17),P<0.01。应用MTD>4 mm诊断EA,正确28例(75.68%),漏诊9例(24.32%);用二尖瓣前瓣根部距心尖的距离与三尖瓣隔瓣根部距心尖距离比(MTR)>1.5诊断EA,正确35例(94.59%),漏诊2例(5.41%);二者间差异有统计学意义(P<0.05)。与存活组比较,死亡组CTR>0.5、G0SE评分>1.0、动脉导管逆向血流、PA/AO<1的例数明显增多,差异有统计学意义(P<0.01)。结论根据MTR>1.5,胎儿超声可比较准确地诊断EA。CTR>0.5、G0SE评分>1.0、动脉导管逆向血流、PA/AO<1是死亡高危因素。

Objective To assess the diagnostic value of Ebstein anomaly(EA)by fetal echocardiography,and to explore the optimal diagnostic criteria and prognostic index.Methods The case group included 37 fetuses with EA diagnosed by prenatal echocardiography,the control group included 37 normal fetuses matched for gestational age,the echocardiographic images and indexes were compared with findings of autopsy and postnatal echocardiography.The correlation between the echocardiographic parameters and the perinatal clinical outcome was evaluated.Results Compared with the control group,the cardiothoracic ratio(CTR)was significantly higher(0.49±0.28)vs(0.28±0.05),P<0.01,mitral valve-tricuspid valve distance(MTD)was significantly higher(7.80±2.39)mm vs(3.80±1.20)mm,P<0.01,the ratio of pulmonary artery diameter to aortic artery diameter(PA/AO)was significantly lower in the case group(0.94±0.23)vs(1.12±0.17),P<0.01.In case group,28 cases(75.68%)were diagnosed correctly,9 cases(24.32%)were miss diagnosed based the diagnostic criterion of MTD>4 mm;35 cases(94.59%)were diagnosed correctly,2 cases(5.41%)were miss diagnosed based the diagnostic criterion of MTR(the distance from the root of the anterior mitral leaflets to the apex/the distance from the root of the tricuspid septal leaflets to the apex)>1.5,there was significantly difference(P<0.05).Compared with survival group,the cases of CTR>0.5,GOSE scores>1.0,PA/AO<1,ductus arteriosus reverse perfusion were more frequently seen in death group(P<0.01).Conclusion EA could be diagnosed accurately by fetal echocardiography based on MTR>1.5.Fetuses with CTR>0.50,the GOSE scores>1.0,PA/AO<1.0,ductus arteriosus reverse perfusion have the highest risk of dying and poor prognostic factors in the perinatal stage.