Abstract
Primary mitochondrial diseases (PMD) are caused by mitochondrial dysfunction due to mutations in nuclear or mitochondrial DNA. Cardiac involvement is present in the majority of patients with cardiomyopathy, arrhythmias, and conduction defects being the most prevalent. Hypertrophic cardiomyopathy is the most frequently encountered cardiomyopathy in PMD but also dilated cardiomyopathy
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