Rebeca Lopes Figueira, Fabian Doktor, Nazgol Moheimani, Giuseppe Lauriti, Jessie Lu, Giuseppe Speziale, Augusto Zani
Hospital for Sick Children. Washington University School of Medicine. University of Leipzig Medical Center. “G. d’Annunzio” University of Chieti-Pescara, “Spirito Santo” Hospital of Pescara.
Canada, United States, Germany and Italy
Pediatric Surgery International
Pediatr Surg Int 2025; 42:
DOI: 10.1007/s00383-025-06247-9
Abstract
Purpose: Pulmonary hypertension secondary to congenital diaphragmatic hernia (CDH-PH) is a major determinant for poor outcomes and high mortality patients with CDH. Identifying risk factors for PH is critical to improve clinical management and optimize treatment approaches. The aim of this study was to investigate factors associated with the development of CDH-PH.
Methods: A systematic review of the literature was registered on PROSPERO and performed according to the PRISMA guidelines. PubMed and Medline were searched for studies published until February 2025 reporting the incidence, mortality and/or risk factors of CDH-PH in human. Studies with overlapping patient populations not meeting inclusion criteria were excluded. CDH-PH incidence was estimated using a proportional meta-analysis. Risk ratios (RRs) for risk factors were calculated using the Mantel-Haenszel method with a randoms effect model in RevMan 5.4. Results are presented as proportions or RRs with 95% confidence intervals (CIs).
Results: Of 11,618 studies, 193 full-text articles were screened, and a total of 59 articles were included. The overall incidence of CDH-PH in infants was 45% (95%CI: 40-50%), with higher risk of death in comparison to CDH newborns without PH [RR 5.04 (3.43-7.39); p < 0.05]. Risk factors for CDH-PH were: pre-operative liver herniation [RR 1.75 (1.48-2.08; p < 0.05], non-left CDH (bilateral and Right-CDH) [RR 1.20 (1.08-1.33); p < 0.05], female sex [RR 1.07 (1.04-1.09); p < 0.05], and congenital heart disease [RR 2.15 (1.48-3.12); p < 0.05].
Conclusions: We found that almost half patients with CDH suffer from PH and that CDH-PH patients have a fivefold higher risk of death in comparison to CDH patients without PH. Although the etiology of CDH-PH remains elusive, the risk factors identified may indirectly indicate that a high degree of mechanical compression on the fetal lungs contributes to vascular remodeling and predisposes to CDH-PH.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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