基于产前诊断的不同侧别先天性膈疝患者临床特征与心脏结构特点研究

Comparative study on clinical characteristics and prenatal heart structures of children with a prenatal diagnosis of left or right congenital diaphragmatic hernia

目的对不同侧别先天性膈疝(congenital diaphragmatic hernia,CDH)患者的临床特征和心脏结构进行对比分析。方法以2009年4月至2019年12月上海交通大学医学院附属新华医院小儿外科收治的产前诊断为CDH的179例患者为研究对象,所有患者采用“产前-产时-产后”程序化多学科综合诊疗模式。对不同侧别CDH患者的产前指标、产时新生儿临床结果、手术相关参数、临床结局以及产前超声心动图上心脏结构参数进行比较。结果179例中,左侧膈疝(left CDH,LCDH)139例(77.7%),归入LCDH组;右侧膈疝(right CDH,RCDH)40例(22.3%),归入RCDH组。179例总体存活率为68.7%(123/179)。RCDH组死亡率高于LCDH组(20.9%vs.45.0%,P=0.034)。RCDH组早产发生率更高(P=0.024),入院1 h内血pH更低(P=0.001)、PaCO_(2)值更高(P=0.002)。LCDH组中,B类缺损占比最多,RCDH组中D类缺损占比最多,两组不同缺损类型占比差异有统计学意义(P=0.007)。41例行产前超声心动图检查的CDH患者中,LCDH患者左心室标准化内径更小[(-2.58±0.77)vs.(-1.60±0.79),P=0.009]、右心室/左心室内径值更大[(1.09±0.09)vs.(0.99±0.07),P=0.015]。LCDH患者中,有肝脏疝入的胎儿右心室/左心室内径值更大[(1.15±0.11)vs.(1.06±0.07),P=0.027]。LCDH患者的左肺动脉标准化内径明显小于右肺动脉标准化内径[(-3.06±1.61)vs.(-2.40±1.43),P=0.041];而在RCDH患者中,上述两项指标差异无统计学意义(P>0.05)。结论产前诊断为RCDH的患者产时情况更危急,膈肌缺损更严重,存活率更低;产前诊断为LCDH的患者左心结构较RCDH患者小,肝脏疝入对左心结构可能有影响。

Objective To compare the clinical features and fetal cardiovascular structures in children with a prenatal diagnosis of left/right congenital diaphragmatic hernia(CDH).Methods From April 2009 to December 2019,a retrospective review was conducted for 179 CDH children.All of them participated in the antenatal-intrapartum-postnatal all-through service mode.Children with a prenatal diagnosis of left CDH(LCDH)and right CDH(RCDH)were compared with regards to prenatal parameters,neonatal outcomes,operation-related parameters,clinical outcomes and cardiac structure parameters on fetal echocardiography(ECHO).Results The involved side was left(n=137,77.7%)and right(n=40,22.3%).A total of 123 CDH children survived with an overall survival rate of 68.7%.The mortality rate of RCDH was higher than that of LCDH(20.9%vs.45.0%,P=0.034).The incidence of preterm delivery was higher(P=0.024),pH value lower(P=0.001)and PaCO_(2) value higher(P=0.002)within 1 hour of admission in RCDH.The proportion of type B defect was greater in LCDH and type D defect predominated in RCDH.Significant inter-group difference existed in the proportion of each type(P=0.007).In 41 fetuses examined by fetal ECHO,left ventricular diameter z-score of LCDH fetus was smaller(P=0.009)and right/left ventricle diameter(RVd/LVd)higher(P=0.027).In LCDH fetuses,Vd/LVd of fetus with liver hernia was higher(P=0.027).Left pulmonary artery diameter z-score of LCDH was significantly smaller than that of RCDH(P<0.007).However,no significant difference existed in RCDH fetus.Conclusion In children with a prenatal diagnosis of RCDH,worse condition at delivery and larger size of diaphragm defect denote a lower survival rate.In LCDH fetuses,the structure of left heart is smaller than that of RCDH fetuses.Hepatic hernia may affect the structure of left heart.