ArticleJournal of the American College of Cardiology2024
Intrahepatic Transcriptomics Differentiate Advanced Fibrosis and Clinical Outcomes in Adults With Fontan Circulation.
Article in Journal of the American College of Cardiology, 2024. 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, 12 citations in OpenAlex.
- Beyond Passive Congestion: The Inflammatory Gut-Liver Axis in Fontan Circulation.Pediatric cardiology · 2026Review
- Single-cell spatial transcriptomics reveals hepatocyte reprogramming in Fontan-associated liver disease.JCI insight · 2026Article
- Multiplexed targeted mRNA profiling of alcohol-related liver disease reveals stage-specific dysregulation of signaling pathways.Molecular biology reports · 2026Article
- The Proteomic Profile of Adults With a Fontan Circulation.JACC. Advances · 2026Article
- NT-proBNP and other blood biomarkers in patients after Fontan Surgery: Prognostic, diagnostic, and management implications.International journal of cardiology. Congenital heart disease · 2026Article
- Prognostic biomarkers in Fontan associated liver disease.Translational pediatrics · 2026Review
- Fontan-Associated Liver Disease: Trends Over Time and Relationship With Outcomes.Journal of the American Heart Association · 2025Article
- The Cardiohepatic Axis in Heart Failure.JACC. Basic to translational science · 2025Review
- Overcoming borders: International cooperation in re-use and re-interpretation of omics data in Fontan circulation.International journal of cardiology. Congenital heart disease · 2025Article
- One Ventricle, 3000 Proteins: Unraveling the Molecular Landscape of the Fontan Circulation.Journal of the American Heart Association · 2025Article
- Comprehensive Multiomic Analysis Reveals Metabolic Reprogramming Underlying Human Fontan-Associated Liver Disease.Journal of the American Heart Association · 2025Article
- Urinary Extracellular Vesicle Analysis Reveals Early Signs of Kidney Inflammation and Damage in Single Ventricle Paediatric Patients After Fontan Operation.International journal of nanomedicine · 2025Article
- Treatment of sinus node dysfunction after the Fontan operation: Time to pick up the pace?Indian pacing and electrophysiology journalArticle
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Authors and funding
19 authors at 1 institution in 1 country.
Funding
Abstract
backgroundThe molecular mechanisms underlying Fontan-associated liver disease (FALD) remain largely unknown.
objectivesThis study aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes.
methodsThis retrospective cohort study included adults with the Fontan circulation. Baseline clinical, laboratory, imaging, and hemodynamic data as well as a composite clinical outcome (CCO) were extracted from medical records. Patients were classified into early or advanced fibrosis. RNA was isolated from formalin-fixed paraffin-embedded liver biopsy samples; RNA libraries were constructed with the use of an rRNA depletion method and sequenced on an Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were performed with the use of DESeq2 and Metascape.
resultsA total of 106 patients (48% male, median age 31 years [IQR: 11.3 years]) were included. Those with advanced fibrosis had higher B-type natriuretic peptide levels and Fontan, mean pulmonary artery, and capillary wedge pressures. The CCO was present in 23 patients (22%) and was not predicted by advanced liver fibrosis, right ventricular morphology, presence of aortopulmonary collaterals, or Fontan pressures on multivariable analysis. Samples with advanced fibrosis had 228 upregulated genes compared with early fibrosis. Samples with the CCO had 894 upregulated genes compared with those without the CCO. A total of 136 upregulated genes were identified in both comparisons and were enriched in cellular response to cytokine stimulus or oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-β signaling pathway, and vasculature development.
conclusionsPatients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis.
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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 OpenQuestion graph.