The effect of critical congenital heart disease on mortality and ECMO utilization in congenital diaphragmatic hernia: An analysis of a contemporary cohort of Children's Hospitals.

INTRODUCTION

The detrimental effect of congenital heart disease (CHD) on congenital diaphragmatic hernia (CDH) survival is well known. However, estimates of survival and of extracorporeal membrane oxygenation (ECMO) utilization in CDH associated with critical congenital heart defects (CCHD) have not been described. Accurate data will enable prognostication for prenatal counseling and possibly expansion of the indications for fetal intervention.

METHODS

The Pediatric Health Information System (PHIS) was queried for all infants born with CDH between 2016 and 2024 using ICD-10 diagnostic codes for CDH, CHD, and CCHD. Pearson chi-square test and logistic regression were used to assess relationships between diagnostic codes and use of ECMO or mortality.

RESULTS

There were 4691 NICU admissions for CDH. Of these, 3580 (76.3%) were also diagnosed with any CHD, and 488 (10.4%) with any CCHD. Patients diagnosed with CDH and CCHD were significantly more likely to die (52%, p < 0.01) and undergo open cardiac repair (34.6%, p < 0.01) upon index admission compared to those with isolated CDH. However, there were no significant differences in the use of ECMO cannulation, duration of ECMO, or likelihood of CDH repair on ECMO.

CONCLUSION

Despite similar rates of ECMO utilization, mortality in this cohort is 50%; more than three times the rate of the non-CCHD cohort. 75% of deaths in infants with CDH and CCHD occurred without ECMO, suggesting that ECMO may have been considered futile. These data have important ramifications for prenatal consultation and perhaps on the role of fetal intervention in this cohort with few other therapeutic options.