Alice Dirick, Marilyne Levy, Kelly Mellul, Maxime Coignard, Naziha Khen-Dunlop, Alexandre Lapillonne Julien Stirnemann, Elsa Kermorvant-Duchemin
Necker-Enfants Malades Hospital.
France
Acta Pediatrica
Acta Pediatr 2025;
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.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Quality of Life Associated with Pulmonary Vascular Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes