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Patterns and Outcomes of Epoprostenol Use in Infants with Congenital Diaphragmatic Hernia Requiring Extracorporeal
Nikhil R Shah1, Keerthi Burgi2, Dimitra M Lotakis1
1Section of Pediatric Surgery, C.S. Mott Children's Hospital, Michigan Medicine, Ann Arbor, MI.
Insights
Epoprostenol use in infants with congenital diaphragmatic hernia (CDH) on extracorporeal life support (ECLS) showed comparable survival rates. However, earlier epoprostenol initiation in these critical pediatric cases may improve outcomes.
Area of Science:
- Neonatal Medicine
- Pediatric Cardiology
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) often leads to severe pulmonary hypertension (PH).
- Extracorporeal life support (ECLS) is a critical intervention for neonates with CDH and respiratory failure.
- Pulmonary hypertension in CDH infants on ECLS presents significant management challenges.
Purpose of the Study:
- To evaluate the use of epoprostenol for managing pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS).
- To assess the impact of epoprostenol on survival and clinical outcomes in this vulnerable infant population.
Main Methods:
- Retrospective review of infants with CDH requiring ECLS between 2013 and 2023.
- Comparison of outcomes between infants who received intravenous epoprostenol and those who did not.
- Analysis of survival rates, ECLS duration, and timing of epoprostenol initiation.
Main Results:
- Of 57 infants, 40 (70.2%) received epoprostenol; survival was comparable between groups (60% vs 64%, P=.23).
- Infants receiving epoprostenol had more severe prenatal indicators of CDH.
- Earlier epoprostenol initiation (median day 6 vs 8) was associated with improved survival (P=.012) and shorter ECLS duration (P=.049).
- Refractory PH was the cause of death in 81% of nonsurvivors.
Conclusions:
- Epoprostenol administration in infants with CDH requiring ECLS is a promising therapeutic strategy.
- Earlier initiation of epoprostenol may be associated with improved survival in this high-risk infant population.
- Further research is warranted to optimize epoprostenol use in CDH infants on ECLS.
Objective:
To describe our experience utilizing epoprostenol for pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) requiring extracorporeal life support (ECLS).
Study Design:
We retrospectively reviewed infants diagnosed with CDH who required ECLS at our institution from 2013 to 2023. Data collected included demographics, disease characteristics, medication administration patterns, and hospital outcomes. We first compared infants who received intravenous epoprostenol and those who did not. Among infants who received epoprostenol, we compared survivors and nonsurvivors. χ² test/Fisher's exact and Mann-Whitney tests were used, with significance defined at P < .05.
Results:
Fifty-seven infants were included; 40 (70.2%) received epoprostenol. Infants receiving epoprostenol had lower observed/expected total fetal lung volume (O/E TFLV) on magnetic resonance imaging (20 vs 26.2%, P = .042) as well as higher prenatal frequency of liver-up (90 vs 64.7%, P = .023) and "severe" classification (67.5 vs 35.3%, P = .007). Survival with and without epoprostenol was comparable (60% vs 64%, P = .23). Of those receiving epoprostenol, both survivors and nonsurvivors had similar prenatal indicators of disease severity. Most (80%) of hernia defects were classified as type C/D and 68% were repaired <72 hours after ECLS cannulation. The median age at initiation of epoprostenol was day of life 6 (IQR: 4, 7) in survivors and 8 (IQR: 7, 16) in nonsurvivors (P = .012). Survivors had shorter ECLS duration (11 vs 20 days, P = .049). Of nonsurvivors, refractory PH was the cause of death for 13 infants (81%).
Conclusions:
In infants with CDH requiring ECLS, addition of epoprostenol appears promising and earlier initiation may affect survival.
