J Perinatol

. 2022 Apr 23. doi: 10.1038/s41372-022-01397-3. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/35461332/

Improved survival for infants with severe congenital diaphragmatic hernia

Jason Gien ^1 2, John P Kinsella ^3 4, Nicholas J Behrendt ^4 5, Michael V Zaretsky ^4 5, Henry L Galan ^4 5, Kenneth W Liechty ^4 6Affiliations expand

• PMID: 35461332
• DOI: 10.1038/s41372-022-01397-3

Abstract

Background: Survival for severe (observed to expected lung-head ratio (O:E LHR) < 25%) congenital diaphragmatic hernia (CDH) remains a challenge (15-25%). Management strategies have focused on fetal endoscopic tracheal occlusion (FETO) and/or extracorporeal membrane oxygenation therapy (ECMO) utilization.

Objective(s): Describe single center outcomes for infants with severe CDH.

Study design: Observational study of 13 severe CDH infants managed with ECMO, a protocolized DR algorithm, and early repair on ECMO with an innovative perioperative anticoagulation strategy.

Results: 13/140 (9.3%) infants met criteria and were managed with ECMO. 77% survived ECMO and 69% survived to discharge. 22% underwent tracheostomy. Median days on mechanical ventilation was 39 days (IQR 22:107.5) and length of stay 135 days (IQR 62.5:211.5). All infants received a gastrostomy tube (GT) and were discharged home on oxygen and pulmonary hypertension (PH) meds.

Conclusion: Survival for infants with severe CDH can be optimized with early aggressive intervention and protocolized algorithms (149).

© 2022. The Author(s), under exclusive licence to Springer Nature America, Inc.