Abstract Title:

Reversal of cardiac hypertrophy with a ketogenic diet in a child with mitochondrial disease and hypertrophic cardiomyopathy.

Abstract Source:

Can J Cardiol. 2020 Apr 28. Epub 2020 Apr 28. PMID: 32360196

Abstract Author(s):

Emilie Deberles, Pascale Maragnes, Marie-José Penniello-Valette, Stéphane Allouche, Michael Joubert

Article Affiliation:

Emilie Deberles


Mitochondrial diseases are rare metabolic disorders which can cause hypertrophic cardiomyopathy. We describe a 3-year-old girl who was diagnosed for mitochondrial disease (mutation m.5559A>G in the mitochondrial-tRNAgene (MT-TW)). Echocardiography showed left ventricular hypertrophy with an enlarged septum (9 mm, Z-score 3.26). Antioxidant supplementation associated with a high-fat ketogenic diet were introduced and improved, as expected, neurological status. In addition, heart parameters improved with a normalization of interventricular septum thickness at the age of six (6 mm, Z-score 1.05). This case report suggests that a ketogenic diet may improve hypertrophic cardiomyopathy in the context of mitochondrial disease.

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