P2C071: Use of a Neonatal Stabilization Pathway for Congenital Diaphragmatic Hernia Improves Outcomes

Purpose/Objectives: Congenital diaphragmatic hernia (CDH) is a rare disorder affecting 2.5 per 10,000 live births. Mortality and morbidity rates remain high despite advances in neonatal critical care including medical management of pulmonary hypertension, ventilatory support, use of extracorporeal membrane oxygenation (ECMO), and timing of operative intervention. The care and resuscitation immediately following delivery of a neonate with CDH varies between neonatologists. The aim of this study was to determine if a CDH early stabilization pathway at a single-center, tertiary pediatric referral center results in improved patient outcomes.

Design/Methods: A retrospective review was conducted at a single-center, tertiary pediatric referral center of neonates diagnosed with and treated for CDH between January 2012 to December 2022. After stakeholder input, a CDH early stabilization pathway was initiated in February 2019. The pathway includes resuscitation goals, respiratory care guidelines, and criteria for escalation to ECMO support. Demographics, CDH characteristics, and in-hospital outcomes were reviewed. Outcomes were compared between the pre- and post-pathway implementation.

Results: A total of 70 infants were identified. The majority were male (62.8%); 86% patients had left sided CDH. Twenty-four infants (34%) required ECMO cannulation with 19 (79%) undergoing repair while on ECMO. The median length of stay was 52 days [IQR: 15 – 44.5], and the in-hospital mortality rate was 24% (17/70) (Figure 1). Survival rates were similar between infants with a left and right sided CDH, 76.8% vs 81.8% (p=1.00), and those with a diagnosis of pulmonary hypertension (PHTN) or no PHTN, 77.5% vs 100%, respectively (p=0.19). A significant difference of survivability was observed between those requiring ECMO and those without, 39.9% vs 98.1% (p < 0.001), and those with and without concomitant congenital heart disease, 28.6% vs 82.2%, respectively (p < 0.001). A survival difference was observed between infants pre- and post-pathway implementation of 66.7% vs 89.3% (p=0.038: OR = 3.6, 95% CI [1.01, 14.70]). No differences were seen between CDH laterality, cardiac disease, PHTN, or ECMO requirement and CDH implementation (Table 1).

Conclusion/Discussion: A neonatal stabilization pathway creates a standard for resuscitation immediately post-delivery in CDH patients. Following the implementation of the pathway, an increase in survival was observed among those infants with CDH. Multiple interventions were implemented simultaneously that may have contributed to this change. Other factors that may have contributed to survivability include CDH severity as dictated by fetal lung to height ratio (LHR) or observed to expected LHR and future studies should assess these factors for their contribution.