ArticleRespiratory research2017
Changes in vasoactive pathways in congenital diaphragmatic hernia associated pulmonary hypertension explain unresponsiveness to pharmacotherapy.
Article in Respiratory research, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 2 of them syntheses that pooled it.
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Who cites it
18 citing papers in PubMed, 2 syntheses or guidelines pooled it, 34 citations in OpenAlex.
- Incidence and risk factors of pulmonary hypertension in neonates with congenital diaphragmatic hernia: a systematic review and meta-analysis.Pediatric surgery international · 2025Pooled it
- Pulmonary hypertension secondary to congenital diaphragmatic hernia: factors and pathways involved in pulmonary vascular remodeling.Pediatric research · 2019Pooled it
- A hemodynamic-driven approach to chronic pulmonary hypertension in congenital diaphragmatic hernia.Journal of perinatology : official journal of the California Perinatal Association · 2026Review
- Pulmonary T2* quantification of fetuses with congenital diaphragmatic hernia: a retrospective, case-controlled, MRI pilot study.Pediatric research · 2026Article
- Hemodynamic precision to guide surgical timing for neonates with congenital diaphragmatic hernia: a narrative review.Journal of perinatology : official journal of the California Perinatal Association · 2025Review
- Inhaled Nitric Oxide in Neonatal Pulmonary Hypertension.Clinics in perinatology · 2024Review
- The cardiopulmonary benefits of physiologically based cord clamping persist for at least 8 hours in lambs with a diaphragmatic hernia.Frontiers in pediatrics · 2024Article
- Hemodynamic management of congenital diaphragmatic hernia: the role of targeted neonatal echocardiography.World journal of pediatric surgery · 2024Review
- Early nitric oxide is not associated with improved outcomes in congenital diaphragmatic hernia.Pediatric research · 2023Article
- Pulmonary vasodilator strategies in neonates with acute hypoxemic respiratory failure and pulmonary hypertension.Seminars in fetal & neonatal medicine · 2022Review
- The Cellular and Molecular Effects of Fetoscopic Endoluminal Tracheal Occlusion in Congenital Diaphragmatic Hernia.Frontiers in pediatrics · 2022Review
- Challenges and Pitfalls: Performing Clinical Trials in Patients With Congenital Diaphragmatic Hernia.Frontiers in pediatrics · 2022Review
- Emerging antenatal therapies for congenital diaphragmatic hernia-induced pulmonary hypertension in preclinical models.Pediatric research · 2021Review
- Congenital diaphragmatic hernia: a narrative review of controversies in neonatal management.Translational pediatrics · 2021Review
- Treatment of pulmonary hypertension during initial hospitalization in a multicenter cohort of infants with congenital diaphragmatic hernia (CDH).Journal of perinatology : official journal of the California Perinatal Association · 2021Article
- A proteome signature of umbilical cord serum associated with congenital diaphragmatic hernia.Nagoya journal of medical science · 2020Article
- Impact of Fgf10 deficiency on pulmonary vasculature formation in a mouse model of bronchopulmonary dysplasia.Human molecular genetics · 2019Article
- Current and emerging pharmacotherapies for the treatment of pulmonary arterial hypertension in infants.Expert opinion on pharmacotherapyReview
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPatients with congenital diaphragmatic hernia (CDH) have structural and functional different pulmonary vessels, leading to pulmonary hypertension. They often fail to respond to standard vasodilator therapy targeting the major vasoactive pathways, causing a high morbidity and mortality. We analyzed whether the expression of crucial members of these vasoactive pathways could explain the lack of responsiveness to therapy in CDH patients.
methodsThe expression of direct targets of current vasodilator therapy in the endothelin and prostacyclin pathway was analyzed in human lung specimens of control and CDH patients.
resultsCDH lungs showed increased expression of both ETA and ETB endothelin receptors and the rate-limiting Endothelin Converting Enzyme (ECE-1), and a decreased expression of the prostaglandin-I
conclusionsTogether, these data demonstrate aberrant expression of targeted receptors in the endothelin and prostacyclin pathway in CDH already early during development. The analysis of this unique patient material may explain why a significant number of patients do not respond to vasodilator therapy. This knowledge could have important implications for the choice of drugs and the design of future clinical trials internationally.
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Registered trials
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