Hypertrophic cardiomyopathy (HCM) has been recently recognized as the most 
common inherited cardiovascular disorder, affecting 1 in 500 adults worldwide. 
HCM is characterized by myocyte hypertrophy resulting in thickening of the 
ventricular wall, myocyte disarray, interstitial and/or replacement fibrosis, 
decreased ventricular cavity volume and diastolic dysfunction. HCM is also the 
most common cause of sudden death in the young. A large proportion of patients 
diagnosed with HCM have mutations in sarcomeric proteins. However, it is unclear 
how these mutations lead to the cardiac phenotype, which is variable even in 
patients carrying the same causal mutation. Abnormalities in calcium cycling, 
oxidative stress, mitochondrial dysfunction and energetic deficiency have been 
described constituting the basis of therapies in experimental models of HCM and 
HCM patients. This review focuses on evidence supporting the role of cellular 
metabolism and mitochondria in HCM.