ArticleScientific reports2020
Fontan-associated liver disease and hepatocellular carcinoma in adults.
Article in Scientific reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 42 citations in OpenAlex.
- Impact of Respiratory Mechanics on Venous Return in Single Ventricle Physiology: A Clinical Imaging Study.JACC. Basic to translational science · 2026Article
- Childhood Liver-Related Abnormalities After Fontan Completion: Evidence from a 20-Year Tertiary Centre Cohort.Pediatric cardiology · 2026Article
- Limited association between liver stiffness and clinical outcomes in Fontan-associated liver disease: a retrospective analysis.International journal of cardiology. Congenital heart disease · 2026Article
- Reduced atrial conduit strain and abnormal diastolic filling are associated with fontan-associated liver disease.The international journal of cardiovascular imaging · 2026Article
- Bile Acid Pool Expansion and Hemodynamic Associations of the Secondary-to-Primary Bile Acid Ratio in Adult Fontan Circulation.Pediatric cardiology · 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
- The use of direct oral anticoagulants in children with congenital and acquired heart disease.Frontiers in cardiovascular medicine · 2026Review
- Link between Metabolic Dysfunction-Associated Steatotic Liver Disease and Cardiovascular Diseases.Research (Washington, D.C.) · 2026Review
- Fontan-Associated Liver Disease (FALD) in the EUROFontan Experience. An Insight into European Awareness.Pediatric cardiology · 2026Observational
- 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
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- Doppler ultrasound and 2D shear wave ultrasound elastography for liver fibrosis evaluation in Fontan-associated liver disease.BMC gastroenterology · 2025Article
- Interventions for adult congenital heart disease.Nature reviews. Cardiology · 2025Review
- Surgical Treatment of Hepatocellular Carcinoma Arising from Fontan-associated Liver Disease: A Report of Three Cases.Internal medicine (Tokyo, Japan) · 2025Review
- Risk of malignancy in adult patients with congenital heart disease: a clinical practice review.Cardiovascular diagnosis and therapy · 2025Review
- Impact of ursodeoxycholic acid treatment on Fontan-associated liver disease.Journal of gastroenterology · 2025Article
- Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management.Frontiers in medicine · 2024Review
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5-5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.
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Registered trials
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.