ArticleHepatology international2026
Platelet slope and long-term clinical outcomes in children and adults with Fontan-associated liver disease.
Article in Hepatology international, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- Article
- Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy.Current cardiology reports · 2026Review
- Critical appraisal of diagnostic criteria and statistical methodology in FALD study.Hepatology international · 2026Article
- Concerns on diagnostic criteria and confounding factors in the study of platelet slope and outcomes in Fontan-associated liver disease.Hepatology international · 2026Article
- Ultrasound Evaluation of Fontan-Associated Liver Disease: A State-of-the-Art Review.Diagnostics (Basel, Switzerland) · 2025Review
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7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesThere is a lack of robust literature describing a relationship between platelet count as a reflection of liver fibrosis and Fontan-associated liver disease (FALD). The present study investigated the serial longitudinal relationship of laboratory tests to cirrhosis and clinical outcomes in patients following Fontan procedure.
methodsThis was a retrospective study of patients with Fontan procedure who underwent laboratory evaluation at least 1 year after surgery. Clinical data, including death, failing Fontan physiology, and heart transplantation, were investigated. Cirrhosis was defined as stage 4 fibrosis on liver biopsy and/or evidence of cirrhosis from imaging. Portal hypertension (PHTN) was calculated using the VAST score (one point each for Varices, Ascites, Splenomegaly, and Thrombocytopenia); VAST score ≥ 2 indicating PHTN features.
resultsAmong 376 patients (184 children and 192 adults), cirrhosis was recorded in 52/376 (13.8%). Platelet counts in those with FALD-associated cirrhosis decreased significantly starting 25, 30 and 35 years after Fontan, compared to the non-cirrhosis group (151 vs. 188; p = 0.01, 134 vs. 174; p = 0.02, and 127 vs. 202 × 10
conclusionsFALD patients with cirrhosis develop decreasing platelet counts 25 years after Fontan procedure. Lower platelets, even if near normal range, can be a marker of cirrhosis in FALD. Cirrhosis with PHTN is an associated with worse heart transplant-free survival, overall survival, and failing Fontan.
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