Observational studyOpen heart2020
Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation.
Observational study in Open heart, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
What it found
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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.
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Who cites it
23 citing papers in PubMed, 37 citations in OpenAlex.
- Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants.Circulation. Cardiovascular imaging · 2022Trial
- Examination of elevated myocardial extracellular volume and exercise capacity in pediatric Fontan patients at moderate altitude.Pediatric radiology · 2026Article
- Unifying and unique roles of non-coding RNA biomarkers in liver and heart fibrosis.Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie · 2026Review
- Imaging findings in Fontan-associated liver disease: Results of a single center study.International journal of cardiology. Congenital heart disease · 2026Article
- Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and Protein-Losing Enteropathy.Current cardiology reports · 2026Review
- Natural Shear Wave Elastography: A Promising Tool to Assess Myocardial Stiffness in Fontan Patients.JACC. Advances · 2026Article
- Long-term survival and complications of Fontan patients: where do we stand?American journal of cardiovascular disease · 2026Review
- Cytokeratin-18 is Elevated Prior to Conventional Measures of Liver Disease in Fontan-Associated Liver Disease.Pediatric cardiology · 2025Article
- T1 mapping of the right ventricle in congenital heart disease: a focus on pediatrics.Pediatric radiology · 2025Review
- Cardiac MRI in patients with Fontan circulation: assessing risk factors for adverse outcomes.Open heart · 2025Article
- Cardiac and Liver Fibrosis Assessed by Multiparametric MRI in Patients with Fontan Circulation.Pediatric cardiology · 2025Article
- Cardiac MRI-Derived Inferior Vena Cava Cross-Sectional Area Correlates with Measures of Fontan-Associated Liver Disease.Pediatric cardiology · 2024Article
- T1 mapping of the myocardium and liver in the single ventricle population.Pediatric radiology · 2023Article
- Heart Failure with Preserved Ejection Fraction in Children.Pediatric cardiology · 2023Review
- Occult Diastolic Dysfunction and Adverse Clinical Outcomes in Adolescents and Young Adults With Fontan Circulation.Journal of the American Heart Association · 2023Observational
- Fontan-associated liver disease: Diagnosis, surveillance, and management.Frontiers in pediatrics · 2023Review
- Therapeutic Approaches in Heart Failure with Preserved Ejection Fraction (HFpEF) in Children: Present and Future.Paediatric drugs · 2022Review
- Invasive Hemodynamic Evaluation of the Fontan Circulation: Current Day Practice and Limitations.Current cardiology reports · 2022Review
- Association of plasma biomarkers and interstitial myocardial fibrosis in fontan population: A machine learning approach.International journal of cardiology. Congenital heart disease · 2022Article
- Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners.US cardiology · 2022Review
Corrections and comments
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Authors and funding
15 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionSingle ventricle diastolic dysfunction and hepatic fibrosis are frequently observed in patients with a Fontan circulation. The relationship between adverse haemodynamics and end-organ fibrosis has not been investigated in adolescents and young adults with Fontan circulation.
methodsProspective observational study of Fontan patients who had a cardiac catheterisation. Cardiac MRI with T1 mapping was obtained to measure extracellular volume (ECV), a marker of myocardial fibrosis. Hepatic magnetic resonance elastography was performed to assess liver shear stiffness. Serum biomarkers of fibrosis including matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs) were measured. Very high ECV was defined as >30% and elevated serum biomarkers as >75th percentile for each biomarker.
results25 Fontan patients (52% female) with mean age of 16.3±6.8 years were included. Mean ECV was 28%±5%. There was a significant correlation between ECV and systemic ventricular end-diastolic pressure (r=0.42, p=0.03) and between ECV and liver stiffness (r=0.45, p=0.05). Patients with elevated ECV demonstrated elevations in MMPs and TIMPs. Similarly, patients with elevated MMPs and TIMPs had greater liver stiffness compared with patients with normal levels of these biomarkers.
conclusionsIn Fontan patients, cardiac magnetic resonance evidence of myocardial fibrosis is associated with diastolic dysfunction, increased liver stiffness and elevated circulating biomarkers of fibrosis. These findings suggest the presence of a profibrotic milieu, with end-organ implications, in some patients with Fontan circulation.
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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.