ArticleJournal of the American Heart Association2017
Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study.
Article in Journal of the American Heart Association, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 114 papers, 2 of them syntheses that pooled it.
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Who cites it
114 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Clinical predictors and noninvasive imaging in Fontan-associated liver disease: A systematic review and meta-analysis.Hepatology communications · 2024Pooled it
- Decreased lipid levels in adult with congenital heart disease: a systematic review and Meta-analysis.BMC cardiovascular disorders · 2023Pooled it
- Characterization of Apixaban Pharmacokinetics/Pharmacodynamics and Dose Assessment in Pediatric Patients with Congenital or Acquired Heart Disease.Clinical pharmacology and therapeutics · 2025Trial
- A Randomized, Controlled Pharmacokinetic and Pharmacodynamics Trial of Ambrisentan After Fontan Surgery.Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies · 2020Trial
- Limited association between liver stiffness and clinical outcomes in Fontan-associated liver disease: a retrospective analysis.International journal of cardiology. Congenital heart disease · 2026Article
- Liver stiffness measured by transient elastography is not predictive of mortality or adverse events in adult patients with Fontan associated liver disease.International journal of cardiology. Congenital heart disease · 2026Article
- Single-cell spatial transcriptomics reveals hepatocyte reprogramming in Fontan-associated liver disease.JCI insight · 2026Article
- Imaging findings in Fontan-associated liver disease: Results of a single center study.International journal of cardiology. Congenital heart disease · 2026Article
- Fontan-associated liver disease in children following surgical correction of congenital heart defects.Clinical and experimental hepatology · 2026Review
- Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy.Current cardiology reports · 2026Review
- Do Hemodynamic and Echocardiographic Derangements After the Fontan Operation Correlate with Severity of Liver Disease on Biopsy?Pediatric cardiology · 2026Article
- Clinical Benefit of Percutaneous Treatment of Fontan Pathway Obstructions.Journal of clinical medicine · 2026Article
- Hepatic cholate clearance as assessed by HepQuant-SHUNT is associated with clinical outcomes in individuals with Fontan circulation.International journal of cardiology. Congenital heart disease · 2026Article
- MRI-derived IVIM-modelled diffusion-weighted imaging does not predict clinical outcomes in fontan-associated liver disease: to monitor or not to monitor the liver?Frontiers in pediatrics · 2026Article
- Fontan-Associated Liver Disease (FALD) in the EUROFontan Experience. An Insight into European Awareness.Pediatric cardiology · 2026Observational
- A One-Dimensional (1D) Computational Fluid Dynamics Study of Fontan-Associated Liver Disease (FALD).International journal for numerical methods in biomedical engineering · 2026Article
- Outcomes and complications of combined heart-liver transplantation in patients with failing Fontan physiology: A systematic review.World journal of transplantation · 2025Article
- Ultrasound Evaluation of Fontan-Associated Liver Disease: A State-of-the-Art Review.Diagnostics (Basel, Switzerland) · 2025Review
- Fontan-Associated Liver Disease: Trends Over Time and Relationship With Outcomes.Journal of the American Heart Association · 2025Article
- Article
54 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
11 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCongestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. METHODS AND
resultsLiver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5 years and mean time from Fontan of 14.9±4.5 years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (
conclusionsIn this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.
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