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The following is a summary of “Patterns and Outcomes of Epoprostenol Use in Infants with Congenital Diaphragmatic Hernia Requiring Extracorporeal Life Support,” published in the September 2024 issue of Pediatrics by Shah et al.

This study evaluates the use of epoprostenol for managing pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) who require extracorporeal life support (ECLS). Researchers conducted a retrospective review of infants with CDH who underwent ECLS at the institution between 2013 and 2023. Data on demographics, disease characteristics, medication administration, and hospital outcomes were collected. They analyzed outcomes by comparing infants who received intravenous epoprostenol with those who did not and further examined survival outcomes among those who received the drug. Statistical analyses were performed using chi-square/Fisher’s exact and Mann-Whitney tests, with significance set at p < 0.05.

The cohort included 57 infants, of whom 40 (70.2%) were administered epoprostenol. Infants treated with epoprostenol had a significantly lower observed/expected total fetal lung volume (O/E TFLV) on MRI (20% vs. 26.2%, p = 0.042) and a higher frequency of prenatal liver-up presentation (90% vs. 64.7%, p = 0.023), as well as a higher proportion classified as “severe” (67.5% vs. 35.3%, p = 0.007). Survival rates were comparable between those who received epoprostenol and those who did not (60% vs. 64%, p = 0.23). Among those receiving epoprostenol, the prenatal indicators of disease severity were similar between survivors and non-survivors. Hernia defects were predominantly classified as Type C/D, with 68% repaired within 72 hours of ECLS cannulation. 

The median age at epoprostenol initiation was day of life 6 (IQR: 4, 7) for survivors and day 8 (IQR: 7, 16) for non-survivors (p = 0.012). Survivors had a shorter duration of ECLS (11 vs. 20 days, p = 0.049), while refractory PH was identified as the cause of death in 13 of 16 non-survivors (81%).

In conclusion, the administration of epoprostenol in infants with CDH on ECLS shows potential benefit, with earlier initiation associated with improved survival. Further studies are needed to confirm these findings and optimize treatment protocols.

Source: sciencedirect.com/science/article/abs/pii/S0022347624003895