TY - JOUR
T1 - Incidence and risk factors of pulmonary hypertension in neonates with congenital diaphragmatic hernia
T2 - a systematic review and meta-analysis
AU - Figueira, Rebeca Lopes
AU - Doktor, Fabian
AU - Moheimani, Nazgol
AU - Lauriti, Giuseppe
AU - Lu, Jessie
AU - Speziale, Giuseppe
AU - Zani, Augusto
N1 - Publisher Copyright:
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2025.
PY - 2026/12
Y1 - 2026/12
N2 - 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.
AB - 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.
KW - Congenital diaphragmatic hernia
KW - Mortality
KW - Prevalence
KW - Pulmonary hypertension
KW - Risk factors
UR - https://www.scopus.com/pages/publications/105022521609
U2 - 10.1007/s00383-025-06247-9
DO - 10.1007/s00383-025-06247-9
M3 - Review article
C2 - 41269355
AN - SCOPUS:105022521609
SN - 0179-0358
VL - 42
JO - Pediatric Surgery International
JF - Pediatric Surgery International
IS - 1
M1 - 23
ER -