ArticleInternational journal of cardiology2013
Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study.
Article in International journal of cardiology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers, 1 of them a synthesis that pooled it.
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Who cites it
76 citing papers in PubMed, 1 synthesis or guideline pooled it, 197 citations in OpenAlex.
- Mortality Outcomes of Combined Heart and Liver Transplantation and Isolated Heart Transplantation Following Fontan Procedures: A Systematic Review and Meta-Analysis.Pediatric transplantation · 2025Pooled it
- Fontan-associated liver disease: The role of liver elastography assessments pre- and post-heart transplant.Journal of pediatric gastroenterology and nutrition · 2026Article
- Beyond Passive Congestion: The Inflammatory Gut-Liver Axis in Fontan Circulation.Pediatric cardiology · 2026Review
- Failing Fontan Following Total Cavopulmonary Connection: Extracardiac Biomarkers Reveal 2 Distinct Phenotypes With Divergent Clinical Courses.Interdisciplinary cardiovascular and thoracic surgery · 2026Article
- Discordant 2D shear wave elastography values in post-Fontan patients with ascites: a potential interpretive pitfall.Journal of ultrasound · 2026Article
- Magnetic Resonance Imaging-Based Psoas Muscle Volume as an Additional Risk Factor for Fontan Failure: A Prospective Cohort Study.Journal of the American Heart Association · 2026Article
- Inter-reader agreement and correlation of VAST, Fontan liver MRI score and MR elastography derived liver stiffness in children and young adults with Fontan-associated liver disease.Abdominal radiology (New York) · 2026Article
- Article
- Fontan-associated liver disease in children following surgical correction of congenital heart defects.Clinical and experimental hepatology · 2026Review
- Diagnostic and Therapeutic Yield of Routine Esophagogastroduodenoscopy in Patients with Fontan Palliation.Pediatric cardiology · 2026Article
- Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy.Current cardiology reports · 2026Review
- Transjugular Liver Biopsy in Children Undergoing Fontan Surgery: Safety and Comparison of Fibrosis Grade with Non-invasive Tests.Pediatric cardiology · 2026Article
- Differences in Prevalence and Severity of Liver Disease Between Lateral Tunnel and Extracardiac Conduit Fontan.JACC. Advances · 2026Article
- Prediction of Fontan failure and correlates of Fontan-associated liver disease severity using machine learning and radiomic features from multi-parametric abdominal MRI.Pediatric radiology · 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
- Platelet slope and long-term clinical outcomes in children and adults with Fontan-associated liver disease.Hepatology international · 2026Article
- Survival by Heart Failure Phenotype in a Fontan Surveillance Population, 2010-2023.Pediatric cardiology · 2026Article
- Fontan-Associated Liver Disease (FALD) in the EUROFontan Experience. An Insight into European Awareness.Pediatric cardiology · 2026Observational
- Fontan-Associated Liver Disease: Trends Over Time and Relationship With Outcomes.Journal of the American Heart Association · 2025Article
- Cytokeratin-18 is Elevated Prior to Conventional Measures of Liver Disease in Fontan-Associated Liver Disease.Pediatric cardiology · 2025Article
16 more citing papers are in PubMed but not listed here.
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Authors and funding
12 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundChronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension in patients post-Fontan operation for single ventricle palliation. The clinical significance of these findings is not clear. We hypothesized that features of portal hypertension would be significantly related to major adverse events.
methodsA retrospective review of 73 adult and pediatric post-Fontan patients referred for a liver evaluation from 2001 to 2011 was performed. The relationship between features of portal hypertension (VAST score ≥2, 1 point each for Varices, Ascites, Splenomegaly or Thrombocytopenia) and a major adverse event (death, need for transplant, or hepatocellular carcinoma) was examined using logistic regression.
results73 post-Fontan patients (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24±11 years, mean interval from Fontan 17±6 years) were included in analysis. Features of portal hypertension (VAST score ≥2) were present in 26 (36%), and there were 19 major adverse events: death (n=12), transplant (n=6), and HCC (n=1). A significant relationship was found between VAST score ≥2 and major adverse events (OR=9.8, 95% CI [2.9-32.7]). After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure, VAST score ≥2 remained significant (OR=9.1, 95% CI [1.4-57.6]).
conclusionFontan patients with features of portal hypertension have a 9-fold increased risk for a major adverse event. Therapies targeted to manage clinical manifestations of portal hypertension, and early referral to heart transplant may help delay major adverse events. Future prospective studies are needed to confirm these findings.
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