胎儿右心发育不良综合征产前超声积分对临床转归的预判分析

Prediction of clinical outcome by prenatal ultrasound score in fetal hypoplastic right heart syndrome

目的:总结胎儿右心发育不良综合征(HRHS)超声心动图特点,探讨产前超声积分与产后结局的关系。方法:回顾性分析2014年1月至2021年3月,在北京安贞医院小儿心脏中心行产前胎儿超声心动图诊断为HRHS胎儿46例为病例组;收集同期孕检,超声孕周匹配且心脏结构及心律/率正常的胎儿86例为对照组。总结胎儿右心发育不良的超声特点,比较两组胎儿右心室内径/左心室内径(RV/LV)、三尖瓣环径/二尖瓣环径(TV/MV)、肺动脉内径/主动脉内径(PA/AO)、右心室(RV)横径Z值、三尖瓣环(TV)Z值、三尖瓣反流程度等参数,计算胎儿右心发育不良严重程度积分追访病例组胎儿预后,对产前积分及产后结局做一致性检验。结果:病例组胎儿RV/LV横径,RV/LV横径Z值,TV/MV,TV Z值,PA/AO均显著低于对照组(P<0.05)。产前积分与TV/MV呈线性关联(OR=-6.8,95%CI:-10.367~-3.233)。产前积分预测TV/MV<0.7(单心室结局)的cut-off值为3.5分(敏感度73.3%,特异度84.6%)。产前积分与生后积分、是否单心室手术一致性一般,产后积分与是否单心室手术一致性良好。结论:胎儿HRHS产前超声积分对产后结局的预判及手术决策的制定有可参考的临床价值。

Objective:To summarize the echocardiographic characteristics of fetal hypoplastic right heart syndrome and to explore the relationship between prenatal ultrasound score and postpartum outcome.Methods:46 fetuses diagnosed as HRHS by prenatal fetal echocardiography in the pediatric cardiovascular center of Beijing Anzhen Hospital from January 2014 to March 2021 were analyzed retrospectively.86 fetuses with normal cardiac structure and rhythm/rate were collected as the control group.We summarize the ultrasonic characteristics of fetal HRHS,compare the parameters of RV/LV transverse diameter,TV/MV,PA/AO,RV transverse diameter Z score,TV Z score and tricuspid regurgitation degree between the two groups,and calculate the score.The fetal prognosis of study group was followed up,the consistency of prenatal score and postpartum outcome was tested.Results:The fetal RV/LV transverse diameter,RV/LV transverse diameter Z score,TV/MV,TV Z score and PA/AO in study group were significantly lower than those in control group(P<0.05).Prenatal score was linearly correlated with TV/MV(OR=-6.8,95%CI:-10.367--3.233).The cut-off value of TV/MV<0.7 predicted by prenatal score was 3.5(sensitivity 73.3%,specificity 84.6%).The consistency is general between prenatal score and postnatal score,whether single-ventricle surgery performed,while the consistency is good between postpartum score and whether single ventricle surgery performed.Conclusions:The prenatal ultrasound score in fetal HRHS is helpful to predict postnatal outcome and operation strategy.