Department of Surgery, Boston Children's Hospital, Boston, MA.
Vascular Biology Program, Boston Children's Hospital, Boston, MA.
Pediatr Crit Care Med. 2020 Jul;21(7):637-646. doi: 10.1097/PCC.0000000000002318.
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.
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.
Neonatal index hospitalization for congenital diaphragmatic hernia.
"Low-risk" congenital diaphragmatic hernia infants born between January 2015 and December 2018.
First postnatal echocardiogram performed within 24 hours from birth.
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.
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.
鉴于人们高度关注提高“高危”先天性膈疝的生存率，有可能忽视了识别“低危”先天性膈疝病例中结局不佳的危险因素的必要性。我们假设，早期心脏功能障碍或严重肺动脉高压是该“低危”先天性膈疝人群不良结局的预测因素。
这是一项使用先天性膈疝研究组登记处数据的回顾性队列研究。“低危”先天性膈疝定义为先天性膈疝研究组缺陷大小 A/B 且无结构性心脏和染色体异常。检查的危险因素包括出生后第 1 次超声心动图上的左心室功能障碍、右心室功能障碍和严重肺动脉高压。主要结局是复合不良事件，定义为死亡、体外膜氧合的使用、出生后第 30 天的氧需求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。
新生儿先天性膈疝指数住院。
2015 年 1 月至 2018 年 12 月出生的“低危”先天性膈疝婴儿。
出生后 24 小时内进行第 1 次超声心动图检查。
确定了 778 名“低危”先天性膈疝患者。分别有 10.8%、20.5%和 57.5%的患者存在左心室功能障碍、右心室功能障碍和严重肺动脉高压。主要结局发生在 21.3%的患者中。3.0%的患者死亡，9.1%的患者使用体外膜氧合。在未调整分析中，所有 3 个危险因素均与主要结局相关。在所有多变量调整方法中，左心室功能障碍和严重肺动脉高压仍然是不良结局的独立预测因素，而右心室功能障碍则不再具有任何影响。
早期左心室功能障碍和严重肺动脉高压是“低危”先天性膈疝婴儿不良结局的独立预测因素。早期识别可能会导致对这一高危队列进行干预，从而改善结局。
