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Pulmonary hypertension secondary to congenital diaphragmatic hernia: factors and pathways involved in pulmonary vascular remodeling.
- Source :
-
Pediatric research [Pediatr Res] 2019 May; Vol. 85 (6), pp. 754-768. Date of Electronic Publication: 2019 Feb 19. - Publication Year :
- 2019
-
Abstract
- Congenital diaphragmatic hernia (CDH) is a severe birth defect that is characterized by pulmonary hypoplasia and pulmonary hypertension (PHTN). PHTN secondary to CDH is a result of vascular remodeling, a structural alteration in the pulmonary vessel wall that occurs in the fetus. Factors involved in vascular remodeling have been reported in several studies, but their interactions remain unclear. To help understand PHTN pathophysiology and design novel preventative and treatment strategies, we have conducted a systematic review of the literature and comprehensively analyzed all factors and pathways involved in the pathogenesis of pulmonary vascular remodeling secondary to CDH in the nitrofen model. Moreover, we have linked the dysregulated factors with pathways involved in human CDH. Of the 358 full-text articles screened, 75 studies reported factors that play a critical role in vascular remodeling secondary to CDH. Overall, the impairment of epithelial homeostasis present in pulmonary hypoplasia results in altered signaling to endothelial cells, leading to endothelial dysfunction. This causes an impairment of the crosstalk between endothelial cells and pulmonary artery smooth muscle cells, resulting in increased smooth muscle cell proliferation, resistance to apoptosis, and vasoconstriction, which clinically translate into PHTN.
- Subjects :
- Animals
Animals, Newborn
Disease Models, Animal
Endothelial Cells drug effects
Endothelial Cells pathology
Endothelial Cells physiology
Female
Hernias, Diaphragmatic, Congenital pathology
Hernias, Diaphragmatic, Congenital physiopathology
Humans
Hypertension, Pulmonary pathology
Hypertension, Pulmonary physiopathology
Infant, Newborn
Myocytes, Smooth Muscle drug effects
Myocytes, Smooth Muscle pathology
Myocytes, Smooth Muscle physiology
Phenyl Ethers toxicity
Pregnancy
Pulmonary Artery drug effects
Pulmonary Artery pathology
Pulmonary Artery physiopathology
Rats
Risk Factors
Vascular Remodeling drug effects
Vascular Remodeling physiology
Hernias, Diaphragmatic, Congenital complications
Hypertension, Pulmonary etiology
Subjects
Details
- Language :
- English
- ISSN :
- 1530-0447
- Volume :
- 85
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Pediatric research
- Publication Type :
- Academic Journal
- Accession number :
- 30780153
- Full Text :
- https://doi.org/10.1038/s41390-019-0345-4