ArticleThe Journal of pediatrics2016
Altered Cholesterol Metabolism and Hypocholesterolemia in Patients with Single Ventricle following Fontan Palliation.
Article in The Journal of pediatrics, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers, 1 of them a synthesis that pooled it.
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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
21 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- 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
- Association Between Hemodynamic Parameters and Gut Microbiota in Fontan Circulation: A Cross-Sectional Study.Journal of the American Heart Association · 2026Article
- Fontan associated protein-losing enteropathy is linked to distinct metabolic and hepatic alterations.Scientific reports · 2026Article
- Insulin Resistance after Fontan Palliation.Pediatric cardiology · 2025Article
- Circulating Biomarkers in Failing Fontan Circulation: Current Evidence and Future Directions.Journal of cardiovascular development and disease · 2025Review
- Systemic Changes in Adults With a Fontan Circulation: Insights From the Plasma Proteome.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
- Blood Biomarkers as a Non-Invasive Method for the Assessment of the State of the Fontan Circulation.Journal of clinical medicine · 2025Review
- Altered Serum Proteins Suggest Inflammation, Fibrogenesis and Angiogenesis in Adult Patients with a Fontan Circulation.International journal of molecular sciences · 2024Article
- Examination of Postoperative Changes in Lipid Profile and Glycemic Markers After Coronary Artery Bypass Graft, Percutaneous Intervention Vs Aortic Valve Replacement Demonstrated a Shift in Risk Factors for Coronary Artery Disease.Journal of multidisciplinary healthcare · 2024Article
- Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy.Metabolites · 2023Review
- Dyslipidemia Among Adults With Congenital Heart Disease.JACC. Advances · 2022Article
- Identification of metabolomic profile related to adult Fontan pathophysiology.International journal of cardiology. Heart & vasculature · 2021Article
- Metabolic profiles identify circulating biomarkers associated with heart failure in young single ventricle patients.Metabolomics : Official journal of the Metabolomic Society · 2021Article
- Fontan-Associated Dyslipidemia.Journal of the American Heart Association · 2021Article
- Long-Term Abnormalities of Lipid Profile After a Single Episode of Sepsis.Frontiers in cardiovascular medicine · 2021Article
- Method comparison of HPLC-ninhydrin-photometry and UHPLC-PITC-tandem mass spectrometry for serum amino acid analyses in patients with complex congenital heart disease and controls.Metabolomics : Official journal of the Metabolomic Society · 2020Article
- Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle.Scientific reports · 2020Article
- Targeted metabolomic analysis of serum amino acids in the adult Fontan patient with a dominant left ventricle.Scientific reports · 2020Article
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveTo assess whether an abnormality in cholesterol absorption or synthesis may be associated with hypocholesterolemia in patients with single ventricle anatomy following Fontan palliation. STUDY
designThis is a cross-sectional study of 21 patients with hypocholesterolemia following Fontan procedure and age/sex-matched healthy controls, with median age of 13.4 (IQR 10.6-16.1) years. Laboratory values of several biomarkers, including phytosterols and 5-α-cholestanol (for cholesterol absorption) and lathosterol (for cholesterol biosynthesis), as well as cholesterol levels, inflammatory markers, and indices of liver function were compared between patients following Fontan procedure and controls.
resultsThe Fontan cohort had significantly lower total cholesterol (mean 117 ± SD 13.9, vs 128 ± 19.2 mg/dL, P = .03) and free cholesterol (35.5 ± 4.5 vs 39.2 ± 5.4 mg/dL, P = .02) compared with control patients. There was an increase in normalized 5-α-cholestanol (1.51 ± 0.6 vs 1.14 ± 0.37 μg/mL, P = .02), and a significantly lower lathosterol/5-α-cholestanol ratio (0.70 ± 0.38 vs 1.11 ± 0.76, P = .04). There was a strong correlation (r = 0.78, P < .0001) between lathosterol and cholesterol levels in the Fontan cohort, not seen in controls (r = 0.47, P = .04). The Fontan cohort also had significantly higher C-reactive protein, transaminases, total bilirubin, and gamma-glutamyl transferase levels.
conclusionsPatients with hypocholesterolemia following Fontan procedure have evidence of increased cholesterol absorption and decreased cholesterol synthesis. As cholesterol absorption efficiency is a regulated process, this finding suggests an upregulation of cholesterol absorption as a result of decreased cholesterol production. In the setting of elevated liver indices and possible inflammation, this finding supports a growing body of data suggesting development of liver disease in patients receiving Fontan.
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Registered trials
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