ArticleThe Journal of pediatrics2017
Fibrosis-Related Gene Expression in Single Ventricle Heart Disease.
Article in The Journal of pediatrics, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
19 citing papers in PubMed, 25 citations in OpenAlex.
- Genetic drivers of congenital cardiac fibrosis.Communications biology · 2026Review
- Exploring Serum Biomarker Levels in Tetralogy of Fallot, Hypoplastic Left Heart Syndrome, and Healthy Children.Pediatric cardiology · 2026Article
- Lung Density Measurement in Fontan Patients Using Zero Echo Time Sequences.Pediatric cardiology · 2026Article
- Pediatric heart failure in the era of cardio-immunology: inflammatory-immune context phenotypes, diagnosis, and targeted management.Frontiers in immunology · 2026Review
- In Vivo Models of Cardiovascular Disease:Biomedicines · 2025Review
- T1 mapping of the right ventricle in congenital heart disease: a focus on pediatrics.Pediatric radiology · 2025Review
- Pediatric heart failure: Current approach and treatment.JHLT open · 2025Article
- Transcoronary study of biomarkers in patients with heart failure: Insights into intracardiac production.ESC heart failure · 2025Article
- Cellular and Transcriptional Landscape of Human Hypoplastic Left Heart Syndrome.Research square · 2025Article
- Management of Pediatric Heart Failure.Korean circulation journal · 2024Review
- Complications and management of functional single ventricle patients with Fontan circulation: From surgeon's point of view.Frontiers in cardiovascular medicine · 2022Review
- Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes.Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance · 2021Article
- Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart.Paediatric drugs · 2021Observational
- Single-Cell RNA Sequencing and Quantitative Proteomics Analysis Elucidate Marker Genes and Molecular Mechanisms in Hypoplastic Left Heart Patients With Heart Failure.Frontiers in cell and developmental biology · 2021Article
- Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.American journal of physiology. Heart and circulatory physiology · 2020Review
- Alteration of cardiolipin biosynthesis and remodeling in single right ventricle congenital heart disease.American journal of physiology. Heart and circulatory physiology · 2020Article
- Pediatric Heart Failure: An Evolving Public Health Concern.The Journal of pediatrics · 2020Article
- Hypoplastic left heart syndrome: From bedside to bench and back.Journal of molecular and cellular cardiology · 2019Review
- Adverse effects of Hif1a mutation and maternal diabetes on the offspring heart.Cardiovascular diabetology · 2018Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
10 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveTo evaluate fibrosis and fibrosis-related gene expression in the myocardium of pediatric subjects with single ventricle with right ventricular failure. STUDY
designReal-time quantitative polymerase chain reaction was performed on explanted right ventricular myocardium of pediatric subjects with single ventricle disease and controls with nonfailing heart disease. Subjects were divided into 3 groups: single ventricle failing (right ventricular failure before or after stage I palliation), single ventricle nonfailing (infants listed for primary transplantation with normal right ventricular function), and stage III (Fontan or right ventricular failure after stage III). To evaluate subjects of similar age and right ventricular volume loading, single ventricle disease with failure was compared with single ventricle without failure and stage III was compared with nonfailing right ventricular disease. Histologic fibrosis was assessed in all hearts. Mann-Whitney tests were performed to identify differences in gene expression.
resultsCollagen (Col1α, Col3) expression is decreased in single ventricle congenital heart disease with failure compared with nonfailing single ventricle congenital heart disease (P = .019 and P = .035, respectively), and is equivalent in stage III compared with nonfailing right ventricular heart disease. Tissue inhibitors of metalloproteinase (TIMP-1, TIMP-3, and TIMP-4) are downregulated in stage III compared with nonfailing right ventricular heart disease (P = .0047, P = .013 and P = .013, respectively). Matrix metalloproteinases (MMP-2, MMP-9) are similar between nonfailing single ventricular heart disease and failing single ventricular heart disease, and between stage III heart disease and nonfailing right ventricular heart disease. There is no difference in the prevalence of right ventricular fibrosis by histology in subjects with single ventricular failure heart disease with right ventricular failure (18%) compared with those with normal right ventricular function (38%).
conclusionsFibrosis is not a primary contributor to right ventricular failure in infants and young children with single ventricular heart disease. Additional studies are required to understand whether antifibrotic therapies are beneficial in this population.
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