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Since the identification of the first pathogenic mutations of mitochondrial DNA in 1988, a plethora of information about human mitochondrial diseases has been brought to light. Not surprisingly, many of these disorders affect the myocardium, because this tissue relies heavily upon oxidative metabolism. This review focuses on disorders of the respiratory chain, the only area of mammalian cellular metabolism under the control of two genomes, nuclear and mitochondrial. Consequently, defects of aerobic synthesis of adenosine triphosphate (ATP) can be due to mutations of either genome. We describe genetic mitochondrial cardiomyopathies and briefly review mouse models and the mitochondrial theory of presbycardia.