Congenital diaphragmatic hernia: exclusion criteria for repair or ECMO?

Abstract

Background: Mortality in congenital diaphragmatic hernia (CDH) approximates 30%. Both severe lung hypoplasia and other anomalies contribute to demise regardless of repair and/or extracorporeal membrane oxygenation (ECMO). We report clinical and physiological parameters for CDH infants not offered repair or ECMO (NoR/ECMO).

Methods: A single center retrospective analysis of 364 CDH infants managed from 01/2003 - 12/2024. We analyzed reasonings to not offer repair or ECMO in 49 infants (13%) across two time epochs. We compared the outcomes of infants that did or did not meet 3 possible lethal lung hypoplasia criteria based on maximum SpO₂ and minimum paCO₂ in the first 24 hours of life.

Results: Concurrent anomalies (n=27, 55%), prematurity < 34 weeks gestation (n=17, 35%), and/or severe lung hypoplasia (n=15, 30%) were the most common reasons for NoR/ECMO; multiple reasons occurred in 19 (39%). There were no differences between epochs for NoR/ECMO. We assessed 3 combinations of highest SpO₂ and lowest paCO₂ in the first 24 hours of life on outcomes. Only 9/364 (2.5%) patients had both highest SpO₂ < 85% and lowest paCO₂ > 75 mmHg; none were offered ECMO or repair. Another 15 infants met one or the other criteria; 4 were offered ECMO and repair with no survivors.

Conclusion: In our center, severe concurrent anomalies, prematurity, and severe lung hypoplasia accounted for 90% of all CDH infants not offered repair or ECMO. Failure to achieve either SpO₂ ≥ 85% and/or paCO₂ ≤ 75 mmHg within the first 24 hours of life despite optimal medical management was uncommon but consistent with lethal lung hypoplasia.