ArticleAmerican journal of physiology. Lung cellular and molecular physiology2023
Congenital heart disease-associated pulmonary dysplasia and its underlying mechanisms.
Article in American journal of physiology. Lung cellular and molecular physiology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 15 citations in OpenAlex.
- Molecular Roadmap of Disrupted Postnatal Lung Development Driven by Increased Pulmonary Blood Flow: Beyond Hemodynamics.JACC. Asia · 2026Article
- The Postnatal Lung Maturation Disrupted by Increased Pulmonary Blood Flow and Its Clinical Implications.JACC. Asia · 2026Article
- A Novel Rapid 3D Tissue-Clearing and Staining Approach for Enteric Neurovascular Imaging and Pathology Applications.Diagnostics (Basel, Switzerland) · 2026Article
- Complement C5a receptor 1 antagonist attenuates alveolar hypoplasia induced by pulmonary hypoperfusion and its underlying mechanisms.Frontiers in immunology · 2026Article
- Increased pulmonary blood flow leads to alveolar dysplasia during the early postnatal developmental stage.Cell & bioscience · 2025Article
- Evaluation of Treatment Effect of Sirolimus on Pediatric Pulmonary Vein Stenosis Using a Neonatal Rat Model.JACC. Basic to translational science · 2025Article
- The complications of cyclosporine a in pediatric use and its effectiveness in treating pediatric congenital heart diseases-a meta analysis in combined with a retrospective clinical study.Frontiers in pharmacology · 2025Article
- Presurgical pulmonary function tests in the first few days of life in neonates with congenital heart disease, a pilot study.Journal of perinatology : official journal of the California Perinatal Association · 2024Article
- Sex Differences in Impacts of Early Gestational and Peri-Adolescent Ozone Exposure on Lung Development in Rats: Implications for Later Life Disease in Humans.The American journal of pathology · 2024Article
- Hemodynamic Melody of Postnatal Cardiac and Pulmonary Development in Children with Congenital Heart Diseases.Biology · 2024Review
- Discovery and functional investigation ofAmerican journal of translational research · 2024Article
- Accurate quantification of pulmonary perfusion ratio in children with congenital heart disease using partial volume corrected 4D flow cardiac magnetic resonance.Frontiers in pediatrics · 2024Article
- Establishing Reference Intervals for Normal Fetal Lung Biological Parameters at 21-40 Weeks of Gestation in the Chinese Population: A Cross-Sectional Study.Diagnostics (Basel, Switzerland) · 2023Article
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Clinical observation indicates that exercise capacity, an important determinant of survival in patients with congenital heart disease (CHD), is most decreased in children with reduced pulmonary blood flow (RPF). However, the underlying mechanism remains unclear. Here, we obtained human RPF lung samples from children with tetralogy of Fallot as well as piglet and rat RPF lung samples from animals with pulmonary artery banding surgery. We observed impaired alveolarization and vascularization, the main characteristics of pulmonary dysplasia, in the lungs of RPF infants, piglets, and rats. RPF caused smaller lungs, cyanosis, and body weight loss in neonatal rats and reduced the number of alveolar type 2 cells. RNA sequencing demonstrated that RPF induced the downregulation of metabolism and migration, a key biological process of late alveolar development, and the upregulation of immune response, which was confirmed by flow cytometry and cytokine detection. In addition, the immunosuppressant cyclosporine A rescued pulmonary dysplasia and increased the expression of the Wnt signaling pathway, which is the driver of postnatal lung development. We concluded that RPF results in pulmonary dysplasia, which may account for the reduced exercise capacity of patients with CHD with RPF. The underlying mechanism is associated with immune response activation, and immunosuppressants have a therapeutic effect in CHD-associated pulmonary dysplasia.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.