Improved mortality rate for congenital diaphragmatic hernia in the modern era of management: 15 year experience in a single institution

Background/purpose: Mortality rates with congenital diaphragmatic hernia (CDH) have remained at approximately 30% for the last 2 decades. Therapies targeting pulmonary hypertension (PHTN) have not been systematically studied in this population, but are increasingly used. We hypothesized that incremental changes in treatments for PHTN have improved mortality for CDH infants.

Methods: Prospective data from 1998 to 2013 on all liveborn CDH patients treated at our institution were retrospectively analyzed. Based on management of PHTN, 4 eras were identified for comparison. Logistic and linear regression were used to compare characteristics. The primary outcome of death prior to discharge was analyzed by multivariable Cox regression modeling.

Results: The study included 192 infants who met inclusion criteria. Length of stay increased, whereas rates of primary repair decreased, suggesting a sicker cohort in the most recent eras. Analysis of mortality across 4 eras showed no difference. By post-hoc analysis, ECMO availability was associated with mortality reduction for eras 3-4 versus 1-2 (HR=0.27, p<0.001).

Conclusions: Improved survival at our institution may be related to recent introduction of ECMO and more aggressive approaches to pulmonary hypertension. Further systematic studies of these PHTN therapies in this specific population are warranted.