ArticleInternational journal of cardiology. Heart & vasculature2021
Identification of metabolomic profile related to adult Fontan pathophysiology.
Article in International journal of cardiology. Heart & vasculature, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 13 citations in OpenAlex.
- 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
- Overcoming borders: International cooperation in re-use and re-interpretation of omics data in Fontan circulation.International journal of cardiology. Congenital heart disease · 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
- Arginine-NO metabolites are associated with morbidity in single ventricle infants undergoing stage 2 palliation.Pediatric research · 2024Article
- A Noninvasive Circulating Signature of Combined Right Ventricular Pressure and Volume Overload in Tetralogy of Fallot/Pulmonary Atresia/Major Aortopulmonary Collateral Arteries.World journal for pediatric & congenital heart surgery · 2024Article
- Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy.Metabolites · 2023Review
- Interstage Single Ventricle Heart Disease Infants Show Dysregulation in Multiple Metabolic Pathways: Targeted Metabolomics Analysis.JACC. Advances · 2023Article
- 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
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMetabolic disorders are important pathophysiologies that can cause multiple organ dysfunction and worsen prognosis in Fontan patients. This study aimed to comprehensively evaluate the metabolomic profile of adult Fontan patients and characterize its pathophysiology in relation to 2 control groups. METHODS AND
resultsWe performed metabolomic analysis of 31 plasma samples using capillary electrophoresis time-of-flight mass spectrometry. This observational cross-sectional study compared plasma metabolites of 14 heterogeneous adult Fontan patients with those of control groups, including 9 patients with congenital heart disease after biventricular repair and 8 normal healthy controls. Fontan patients exhibited significant differences in intermediate metabolite concentrations related to glycolysis, the tricarboxylic acid (TCA) cycle, and the urea cycle. The plasma concentrations of lactic acid, 2-oxoglutarate, isocitric acid, malic acid,
conclusionsThe present findings suggest that abnormalities in the TCA cycle and amino acid metabolism are distinguishing features in the pathophysiology of Fontan patients. Future metabolomic studies will assist in developing biomarkers for the early prediction of "silent" Fontan pathophysiologies.
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