Objectives: 

Given significant focus on improving survival for “high-risk” congenital diaphragmatic hernia, there is the potential to overlook the need to identify risk factors for suboptimal outcomes in “low-risk” congenital diaphragmatic hernia cases. We hypothesized that early cardiac dysfunction or severe pulmonary hypertension were predictors of adverse outcomes in this “low-risk” congenital diaphragmatic hernia population.

Design: 

This is a retrospective cohort study using data from the Congenital Diaphragmatic Hernia Study Group registry. “Low-risk” congenital diaphragmatic hernia was defined as Congenital Diaphragmatic Hernia Study Group defect size A/B without structural cardiac and chromosomal anomalies. Examined risk factors included left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension on the first postnatal echocardiogram. The primary outcome was composite adverse events, defined as either death, extracorporeal membrane oxygenation utilization, oxygen requirement on day 30 of life, or hospitalization greater than or equal to 8 weeks. Multivariable adjustment was performed with logistic regression and inverse probability weighting.

Setting: 

Neonatal index hospitalization for congenital diaphragmatic hernia.

Patients: 

“Low-risk” congenital diaphragmatic hernia infants born between January 2015 and December 2018.

Interventions: 

First postnatal echocardiogram performed within 24 hours from birth.

Measurements and Main Results: 

Seven-hundred seventy-eight patients were identified as “low-risk” congenital diaphragmatic hernia. Left ventricular dysfunction, right ventricular dysfunction, and severe pulmonary hypertension were present in 10.8%, 20.5%, and 57.5%, respectively. The primary outcome occurred in 21.3%. Death occurred in 3.0% and 9.1% used extracorporeal membrane oxygenation. On unadjusted analysis, all three risk factors were associated with the primary outcome. On all multivariable adjustment methods, left ventricular dysfunction and severe pulmonary hypertension remained significant predictors of adverse outcomes while right ventricular dysfunction no longer demonstrated any effect.

Conclusions: 

Early left ventricular dysfunction and severe pulmonary hypertension are independent predictors of adverse outcomes among “low-risk” congenital diaphragmatic hernia infants. Early recognition may lead to interventions that can improve outcome in this at-risk cohort.

中文翻译：

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早期左心室功能障碍和严重的肺动脉高压可预测“低风险”先天性ph肌疝的不良结果。

目标： 

由于高度重视提高“高危”先天性diaphragm肌疝的生存率，因此有可能会忽略识别“低风险”先天性diaphragm肌疝病例中次优结局的危险因素的需求。我们假设在这种“低风险”的先天性diaphragm肌疝患者中，早期心脏功能障碍或严重的肺动脉高压是不良结局的预兆。

设计： 

这是一项回顾性队列研究，使用先天性ph肌疝研究组注册表中的数据。“低危”先天性diaphragm肌疝被定义为先天性ph肌疝研究组缺损大小A / B，无结构性心脏和染色体异常。在出生后的第一张超声心动图上检查的危险因素包括左心室功能障碍，右心室功能障碍和严重肺动脉高压。主要结局为复合不良事件，定义为死亡，体外膜氧合利用率，生命30天的需氧量或住院时间大于或等于8周。采用逻辑回归和逆概率加权进行多变量调整。

设置： 

先天性diaphragm肌疝的新生儿指数住院治疗。

耐心： 

2015年1月至2018年12月出生的“低危”先天性diaphragm肌疝婴儿。

干预措施： 

出生后24小时内首次进行产后超声心动图检查。

测量和主要结果： 

780例患者被确定为“低危”先天性diaphragm肌疝。左心功能不全，右心功能不全和严重的肺动脉高压分别占10.8％，20.5％和57.5％。主要结果发生率为21.3％。使用体外膜氧合发生死亡的发生率为3.0％和9.1％。在未经调整的分析中，所有三个风险因素均与主要结局相关。在所有多变量调整方法中，左心功能不全和严重肺动脉高压仍然是不良结局的重要预测指标，而右心功能不全 不再显示任何效果。

结论： 

早期左室功能障碍和严重肺动脉高压是“低危”先天性diaphragm肌疝婴儿不良后果的独立预测因子。尽早识别可能会导致可以改善这一高危人群结果的干预措施。