Perspective of Mitochondrial Cardiology

Myocardial mitochondria are responsible for supplying the necessary energy to maintain continuous function of the heart. Mitochondrial abnormalities encompass dysfunction in various metabolic processes, including the respiratory system, oxidative phosphorylation, β-oxidation of fatty acids, mitochondrial dynamics, and ion homeostasis. Mitochondrial dysfunction signals the initiation and progression of pathophysiological changes in the heart. Mitochondrial cardiomyopathies are invariably associated with depression in ATP production and reduced coenzyme Q10 content in the heart. The minimally invasive high-resolution respirometry (HRR) technique allows detection of even the slightest systemic changes in mitochondrial function within isolated platelets of patients with pathological cardiac activity. The application of both HRR and the method for determining endogenous coenzyme Q10 levels opens new avenues for early diagnosis and targeted therapy of dysfunctional mitochondria in the heart. There is a real challenge in developing mitochondrial transplantation as a therapy of heart disease with mitochondrial abnormalities.