Key result
Children with DCM and HF show ~3-fold higher total acylcarnitines, indicating down-regulated fatty acid oxidation.
Why the study?
Heart failure in adults involves reduced fatty acid oxidation and accumulation of toxic lipid derivatives, but data concerning metabolic modifications in pediatric heart failure were lacking.
Do children with dilated cardiomyopathy and heart failure have altered acylcarnitine profiles compared to healthy controls?
Case-Control (n=17)
Do children with dilated cardiomyopathy and heart failure have altered acylcarnitine profiles compared to healthy controls?
Children with heart failure show significant accumulation of acylcarnitines, indicating down-regulation of fatty acid oxidation that correlates with disease severity.
Answers are grounded in this paper and cite their sources.
Acylcarnitine accumulation associated with pediatric DCM-HF; hypothesis-generating for metabolic therapies and requires prospective validation.
AIMS: Heart failure in adults is characterized by reduction of long-chain fatty acid oxidation in favour of carbohydrate metabolism. This adaptive phenomenon becomes maladaptive because energy conversion decreases and lipid toxic derivatives known to impair cardiac function are accumulating. No data are available concerning metabolic modification in heart failure in children. METHODS AND RESULTS: In order to evaluate the fatty acid oxidation in children suffering from heart failure, acylcarnitine profiles on dried blood spots were obtained from children under 16 years old with dilated cardiomyopathy and clinical heart failure (DCM-HF) and control children. Nine children were included in the DCM-HF group and eight in the control group. Acylcarnitine profiles revealed a significant 3.1-fold increase of total acylcarnitines (sum of C3 to C18 acylcarnitine species) in DCM-HF children compared with controls. This result persisted considering the sum of long-chain acylcarnitines (sum of C14 to C18 species), medium-chain acylcarnitines (sum of C8 to C12 species), and short-chain acylcarnitines (sum of C3 to C6 species), respectively, 2.0-, 2.6-, and 1.9-fold increase compared with the control group. A significant linear correlation was found between left ventricular dilatation or ejection fraction and acylcarnitines accumulation. Finally, acylcarnitine ratio C16OH/C16 and C18OH/C18 enhanced in the DCM-HF group, suggesting a diminution of the long-chain hydroxyl acyl-CoA dehydrogenase activity. CONCLUSIONS: Our results suggest down-regulation of fatty acid oxidation in children with heart failure. Such lipidomic alteration could worsen heart function and may suggest considering a metabolic treatment of heart failure in children.
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Issa et al. (2023) conducted a case-control in Dilated cardiomyopathy and clinical heart failure (n=17). Acylcarnitine profiling vs. Control children was evaluated on Total acylcarnitines (sum of C3 to C18 acylcarnitine species) (3.1-fold increase). Children with dilated cardiomyopathy and heart failure had a significant 3.1-fold increase in total acylcarnitines compared with controls, indicating down-regulated fatty acid oxidation.
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