Research ArticleARRHYTHMOGENIC CARDIOMYOPATHY

Elevated plasma β-hydroxybutyrate predicts adverse outcomes and disease progression in patients with arrhythmogenic cardiomyopathy

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Science Translational Medicine  12 Feb 2020:
Vol. 12, Issue 530, eaay8329
DOI: 10.1126/scitranslmed.aay8329

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Insight from metabolites

Some individuals with arrhythmogenic cardiomyopathy (AC) can live for decades without symptoms, and reliable and specific markers of disease progression remain lacking. Song and colleagues studied metabolic markers in the plasma and hearts of individuals with AC and their relatives. They found elevated plasma ketones, specifically β-hydroxybutyrate, in affected individuals and relatives suspected of having AC. Metabolomics using patient cells and a mouse model of the disease revealed that cardiomyocytes exhibited de novo ketogenesis with higher fatty acid oxidation that favored medium-chain fatty acid substrates. Individuals with early AC with major adverse cardiac events had elevated plasma β-hydroxybutyrate, suggesting that this may be a useful marker of disease progression.

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