目的 探讨先天性膈疝（CDH）的死亡危险因素。方法 收集2017年1月至2021年2月收治的CDH患儿的临床资料,分析影响CDH患儿死亡的危险因素。结果 纳入68例CDH患儿,男42例、女26例,中位胎龄39.0（37.5~39.4）周,平均出生体质量（2 996.9±549.8）g,PICU住院时间19.2（5.3~28.0）d,有创通气时间9.1（3.9~13.9）d。死亡组23例（33.8%）、存活组45例。手术59例,手术后死亡14例（23.7%）。左侧膈疝54例,右侧膈疝14例。与存活组比较,死亡组胎龄较小,出生体质量较低,PICU住院和有创机械通气时间较短,oeLHR较小,pH值较低,1分钟Apgar评分≤7和肝脏疝入比例较高,肺动脉压力和氧合指数（OI）较高,差异均有统计学意义（P<0.05）。二分类logistic回归分析显示,OI及肝脏疝入为CDH死亡的独立危险因素（P<0.05）。结论 CDH有较高的病死率,OI越大,死亡的可能性越高;肝脏疝入患儿发生死亡的风险增高。

Objective To investigate the death risk factors of congenital diaphragmatic hernia (CDH). Methods The clinical data of children with CDH admitted from January 2017 to February 2021 were collected to analyze the risk factors for death of children with CDH. Results A total of 68 children (42 boys and 26 girls) with CDH were included. The median gestational age was 39.0 (37.5~39.4) weeks, the average birth weight was (2996.9±549.8) g, the PICU hospital stay was 19.2 (5.3-28.0) days and the invasive ventilation duration was 9.1 (3.9-13.9) days. There were 23 cases (33.8%) in the death group and 45 cases in the survival group. Fifty-nine cases underwent surgery, and 14 cases died after operation (23.7%). There were 54 cases of left diaphragmatic hernia and 14 cases of right diaphragmatic hernia. Compared with the survival group, the death group had smaller gestational age, lower birth weight, shorter PICU hospitalization and invasive mechanical ventilation time, lower observed/expected lung to head ratio, lower pH value, higher proportion of low Apgar scores (1-minute Apgar score ≤7) and liver herniation, higher pulmonary artery pressure and oxygenation index (OI), and the differences were statistically significant (P<0.05). Binary logistic regression analysis showed that OI and hepatic herniation were independent risk factors for death of CDH children (P<0.05). Conclusions CDH has a higher mortality and the risk of death increases with the increase of OI. Children with liver herniation had increased risk of death.