Acta Paediatr

. 2025 Feb 6.

 doi: 10.1111/apa.70019. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/39912339/

Infants With a Congenital Diaphragmatic Hernia Had Favourable Pulmonary Hypertension Outcomes at 1 Year of Age

Alice Dirickx ¹, Marilyne Levy ², Kelly Mellul ¹, Maxime Coignard ^1 3, Naziha Khen-Dunlop ⁴, Alexandre Lapillonne ^1 3, Julien Stirnemann ^3 5, Elsa Kermorvant-Duchemin ^1 3

Affiliations Expand

• PMID: 39912339
• DOI: 10.1111/apa.70019

Abstract

Aim: Pulmonary hypertension is frequent in neonates with a congenital diaphragmatic hernia, but long-term data have been scarce. Our aim was to examine its prevalence, evolution and management and identify factors associated with its persistence.

Methods: This French retrospective cohort study compared the characteristics of neonates who had persistent pulmonary hypertension, or died with it, and those with normalised pulmonary pressures at 1 month and 1 year of age.

Results: Most (92%) of the 88 neonates we studied underwent surgery. Two-thirds (67%) had preoperative pulmonary hypertension and they included 10 of the 11 who died after surgery. Pulmonary hypertension resolved after a median of 31 days in the 70 who were discharged alive and 27% required prolonged sildenafil treatment. At 1 year, 6 (9%) of the 65 children with echocardiographic data available still had elevated pulmonary pressures. Preoperative pulmonary hypertension, associated malformations and longer invasive ventilation were independently associated with persistent pulmonary hypertension at 1 month. Only prolonged invasive ventilation remained significant at 1 year.

Conclusion: Despite its high initial prevalence and impact on mortality, pulmonary hypertension resolved within weeks of surgery in the surviving neonates. The duration of invasive ventilation may have been a key factor in its persistence.

Keywords: congenital diaphragmatic hernia; foetal tracheal occlusion; pulmonary hypertension; sildenafil.

© 2025 The Author(s). Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.