ArticleScientific reports2020
Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle.
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 28 papers, 2 of them syntheses that pooled it.
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The trial behind it
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Who cites it
28 citing papers in PubMed, 2 syntheses or guidelines pooled it, 35 citations in OpenAlex.
- The Metabolic Signature of Cardiorespiratory Fitness: A Systematic Review.Sports medicine (Auckland, N.Z.) · 2022Pooled it
- Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review.Frontiers in cardiovascular medicine · 2022Pooled it
- NT-proBNP and other blood biomarkers in patients after Fontan Surgery: Prognostic, diagnostic, and management implications.International journal of cardiology. Congenital heart disease · 2026Article
- Fontan associated protein-losing enteropathy is linked to distinct metabolic and hepatic alterations.Scientific reports · 2026Article
- Metabolomic Markers of Left Ventricular Structure, Diastolic Function, and Risk of Coronary Heart Disease: A Longitudinal Study in American Indian Individuals.Journal of the American Heart Association · 2026Article
- Untargeted Serum Proteomics in the Fontan Circulation Reveals Three Distinct Molecular Signatures of Fontan Physiology with CYB5R3 Among Key Proteins.International journal of molecular sciences · 2026Article
- Circulating Biomarkers in Failing Fontan Circulation: Current Evidence and Future Directions.Journal of cardiovascular development and disease · 2025Review
- Endothelial dysfunction in single ventricle physiology and the Fontan circulation - What lies ahead.International journal of cardiology. Congenital heart disease · 2025Review
- Methylated Arginine Metabolites as Biomarkers for Clinical Status and Response to Type 5 Phosphodiesterase Inhibition in Patients With Fontan Circulation.Journal of the American Heart Association · 2025Article
- Comprehensive Multiomic Analysis Reveals Metabolic Reprogramming Underlying Human Fontan-Associated Liver Disease.Journal of the American Heart Association · 2025Article
- Gut microbiota community and metabolic profiles in direct total cavopulmonary connection and Fontan circulation: a cross-sectional study in the single center.Frontiers in microbiology · 2025Article
- The attenuated hepatic clearance of propionate increases cardiac oxidative stress in propionic acidemia.Basic research in cardiology · 2024Article
- Plasma metabolomic and lipidomic profiles accurately classify mothers of children with congenital heart disease: an observational study.Metabolomics : Official journal of the Metabolomic Society · 2024Observational
- Altered Serum Proteins Suggest Inflammation, Fibrogenesis and Angiogenesis in Adult Patients with a Fontan Circulation.International journal of molecular sciences · 2024Article
- Single-cell multiomics guided mechanistic understanding of Fontan-associated liver disease.Science translational medicine · 2024Article
- Review
- Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy.Metabolites · 2023Review
- Cardiac Transcriptome Remodeling and Impaired Bioenergetics in Single-Ventricle Congenital Heart Disease.JACC. Basic to translational science · 2023Article
- Circulating Reactive Oxygen Species in Adults with Congenital Heart Disease.Antioxidants (Basel, Switzerland) · 2022Article
- Metabolomics: A New Tool in Our Understanding of Congenital Heart Disease.Children (Basel, Switzerland) · 2022Review
Corrections and comments
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Authors and funding
13 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Growing interest lies in the assessment of the metabolic status of patients with a univentricular circulation after Fontan operation, especially in changes of amino acid metabolism. Using targeted metabolomic examinations, we investigated amino acid metabolism in a homogeneous adult Fontan-patient group with a dominant left ventricle, seeking biomarker patterns that might permit better understanding of Fontan pathophysiology and early detection of subtle ventricular or circulatory dysfunction. We compared serum amino acid levels (42 analytes; AbsoluteIDQ p180 kit, Biocrates Life Sciences, Innsbruck, Austria) in 20 adult Fontan patients with a dominant left ventricle and those in age- and sex-matched biventricular controls. Serum concentrations of asymmetric dimethylarginine, methionine sulfoxide, glutamic acid, and trans-4-hydroxyproline and the methionine sulfoxide/methionine ratio (Met-SO/Met) were significantly higher and serum concentrations of asparagine, histidine, taurine, and threonine were significantly lower in patients than in controls. Met-SO/Met values exhibited a significant negative correlation with oxygen uptake during exercise. The alterations in amino acid metabolome that we found in Fontan patients suggest links between Fontan pathophysiology, altered cell energy metabolism, oxidative stress, and endothelial dysfunction like those found in biventricular patients with congestive heart failure. Studies of extended amino acid metabolism may allow better understanding of Fontan pathophysiology that will permit early detection of subtle ventricular or circulatory dysfunction in Fontan patients.
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