SEARCHNARRATIVE_REVIEWcardiology, pediatrics

narrative_review·cardiology, pediatrics, clinical nutrition, preclinical research·PMC10456471

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

Metabolites · 4 authors, 5 centres

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TL;DR

A major limitation noted is that most studies were conducted on small patient cohorts, and large-scale multicenter studies are needed to validate these metabolic impairments and explore the therapeutic potential of ketosis.

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narrative_review

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cardiology

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ORIGINAL PAPER

PMC10456471

Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy

Metabolites

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ABSTRACT

Abstract Most studies on single ventricle (SV) circulation take a physiological or anatomical approach. Although there is a tight coupling between cardiac contractility and metabolism, the metabolic perspective on this patient population is very recent. Early findings point to major metabolic disturbances, with both impaired glucose and fatty acid oxidation in the cardiomyocytes. Additionally, Fontan patients have systemic metabolic derangements such as abnormal glucose metabolism and hypocholesterolemia. Our literature review compares the metabolism of patients with a SV circulation after Fontan palliation with that of patients with a healthy biventricular (BV) heart, or different subtypes of a failing BV heart, by Pubmed review of the literature on cardiac metabolism, Fontan failure, heart failure (HF), ketosis, metabolism published in English from 1939 to 2023. Early evidence demonstrates that SV circulation is not only a hemodynamic burden requiring staged palliation, but also a metabolic issue with alterations similar to what is known for HF in a BV circulation. Alterations of fatty acid and glucose oxidation were found, resulting in metabolic instability and impaired energy production. As reported for patients with BV HF, stimulating ketone oxidation may be an effective treatment strategy for HF in these patients. Few but promising clinical trials have been conducted thus far to evaluate therapeutic ketosis with HF using a variety of instruments, including ketogenic diet, ketone esters, and sodium-glucose co-transporter-2 (SGLT2) inhibitors. An initial trial on a small cohort demonstrated favorable outcomes for Fontan patients treated with SGLT2 inhibitors. Therapeutic ketosis is worth considering in the treatment of Fontan patients, as ketones positively affect not only the myocardial energy metabolism, but also the global Fontan physiopathology. Induced ketosis seems promising as a concerted therapeutic strategy. Keywords: biventricular, heart failure, nutrition, Fontan, ketones, ketogenic therapy, metabolism, single ventricle

CITATION EXPORT

David Renaud, Sabine Scholl-Bürgi, Daniela Karall et al.. Comparative Metabolomics in Single Ventricle Patients after Fontan Palliation: A Strong Case for a Targeted Metabolic Therapy. Metabolites. (2023). https://doi.org/10.3390/metabo13080932

AUTHORS

4 · 5 CENTRES

David Renaud, Sabine Scholl-Bürgi, Daniela Karall, Miriam Michel

1Fundamental and Biomedical Sciences, Paris-Cité University, 75006 Paris, France
2Health Sciences Faculty, Universidad Europea Miguel de Cervantes, 47012 Valladolid, Spain
3Fundacja Recover, 05-124 Skrzeszew, Poland
4Department of Child and Adolescent Health, Division of Pediatrics I—Inherited Metabolic Disorders, Medical University of Innsbruck, 6020 Innsbruck, Austria
5Department of Child and Adolescent Health, Division of Pediatrics III—Cardiology, Pulmonology, Allergology and Cystic Fibrosis, Medical University of Innsbruck, 6020 Innsbruck, Austria