ArticleThe Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation2015
Portal and centrilobular hepatic fibrosis in Fontan circulation and clinical outcomes.
Article in The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
32 citing papers in PubMed, 2 syntheses or guidelines pooled it, 110 citations in OpenAlex.
- Clinical predictors and noninvasive imaging in Fontan-associated liver disease: A systematic review and meta-analysis.Hepatology communications · 2024Pooled it
- Effect of liver abnormalities on mortality in Fontan patients: a systematic review and meta-analysis.BMC cardiovascular disorders · 2024Pooled it
- Two-dimensional shear wave elastography in Fontan-associated liver disease: a narrative review.Abdominal radiology (New York) · 2026Review
- Single-cell spatial transcriptomics reveals hepatocyte reprogramming in Fontan-associated liver disease.JCI insight · 2026Article
- 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
- Correlation of Liver Fibrosis on Ultrasound Elastography and Liver Biopsy After Fontan Operation: Is Non-invasive Always Better?Pediatric cardiology · 2026Article
- Fontan-Associated Liver Disease (FALD) in the EUROFontan Experience. An Insight into European Awareness.Pediatric cardiology · 2026Observational
- Efficacy of Magnetic Resonance Elastography in Fontan-Associated Liver Disease.JGH open : an open access journal of gastroenterology and hepatology · 2025Article
- Non-invasive fibrosis tools lack clinical utility for identifying advanced fibrosis in Fontan-associated liver disease: a retrospective cohort study.BMJ open gastroenterology · 2025Article
- The changing landscape of multimodality imaging in congenital heart disease: white paper.European heart journal. Imaging methods and practice · 2025Review
- The Cardiohepatic Axis in Heart Failure.JACC. Basic to translational science · 2025Review
- Fontan-Associated Liver Disease.Seminars in liver disease · 2025Review
- Association of liver biopsy pathology on outcome of patients undergoing heart transplantation.JHLT open · 2025Article
- Transfemoral-Transcaval Liver Biopsy (TFTC) and Transjugular Liver Biopsy (TJLB) in Patients with Fontan-Associated Liver Disease (FALD).Cardiovascular and interventional radiology · 2024Article
- Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management.Frontiers in medicine · 2024Review
- Mitochondrial dysfunction is associated with the severity of liver fibrosis in patients after the Fontan operation.Journal of cellular and molecular medicine · 2024Article
- Fontan-associated liver disease.Clinical liver disease · 2023Review
- Assessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation.European radiology · 2023Article
- Fontan-associated liver disease: Diagnosis, surveillance, and management.Frontiers in pediatrics · 2023Review
Corrections and comments
- Commented on by
Authors and funding
11 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe Fontan operation redirects venous blood flow directly to the pulmonary circulation in subjects with single ventricle anatomy. Congestive hepatopathy and cirrhosis have been described in subjects with Fontan circulation, but the prevalence of and predictors for liver disease remain unknown.
methodsWe performed a retrospective study of liver histopathology in Fontan subjects who had liver biopsy or autopsy. All specimens were graded using a pre-determined protocol. Additional data were collected through chart review. Among 68 subjects, specimens were obtained at a median age of 23.2 years (range 5.0 to 52.7 years). Median time since Fontan was 18.1 years (range 1.2 to 32.7 years).
resultsCentrilobular fibrosis was seen in every specimen, with 41.2% showing Grade 4 centrilobular fibrosis. Portal fibrosis was seen in 82.3% of specimens, with 14.7% showing cirrhosis. Megamitochondria were seen in 58.8% of specimens. Centrilobular fibrosis grade was greater in those with a dominant left or right ventricle than in those with a combined right and left systemic ventricle (p = 0.008). Portal fibrosis grade correlated with alkaline phosphatase (p = 0.04) and mode of biopsy (p = 0.02). Neither centrilobular fibrosis nor portal fibrosis grade was predictive of transplant-free survival or overall survival.
conclusionsIndividuals with Fontan physiology have a high prevalence of hepatic fibrosis. Signs and symptoms of liver disease did not predict histopathologic findings. Few risk factors for advanced disease were identified. Histopathology findings did not predict transplant-free survival. The role of liver biopsy in this population remains uncertain.
Indexed as
Identifiers
What OpenQuestion holds
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.