【摘要】 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）、PaCO₂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。更多还原

【Abstract】 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 PaCO₂ 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.更多还原