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Improved survival for infants with severe congenital diaphragmatic hernia

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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).

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Data availability

Data relevant to this manuscript is presented in the main manuscript and if requested will be made available to referees and to readers promptly upon request.

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Acknowledgements

The patients who trusted the Colorado Fetal Care Center and CDH Team at Children’s Hospital Colorado with their care.

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Authors and Affiliations

Authors

Contributions

JG was responsible for conception, acquisition, analysis and interpretation of data for the work. JG wrote the first draft of the manuscript, is agreeable to be accountable for all aspects of the work related to the accuracy or integrity of the work and gave approval for the final version to be published. JPK was responsible for conception and interpretation of data for the work. JK reviewed the initial manuscript draft and provided feedback revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. NJB was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. MVZ was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. HLG was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published. KWL was responsible for conception and interpretation of data for the work. He reviewed the initial manuscript draft and provided feedback, revising it critically for important intellectual content. He is agreeable to be accountable for all aspects related to the accuracy or integrity of the work and gave approval for the final version to be published.

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Correspondence to Jason Gien.

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The authors declare no competing interests.

Ethics approval and consent to participate

Colorado Institutional review board (COMIRB) approval was obtained with a waiver of consent. The study was performed in accordance with the Declaration of Helsinki.

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Gien, J., Kinsella, J.P., Behrendt, N.J. et al. Improved survival for infants with severe congenital diaphragmatic hernia. J Perinatol 42, 1189–1194 (2022). https://doi.org/10.1038/s41372-022-01397-3

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