Ultrastructural characteristics of myocardial mitochondria in hypertrophic cardiomyopathy with diffuse-generalized phenotype

• Kozlova M.A.
• Areshidze D.A.
• Chernikov V.P.
• Motreva A.P.
• Evseev E.P.
• Elena V. Zaklyazminskaya
• Dzemeshkevich S.L.

Abstract

Background. Hypertrophic cardiomyopathy (HCM) is a common genetic myocardial pathology. Studies have shown the relationship of the severity of histopathologic changes in HCM with clinical manifestations and the risk of life-threatening arrhythmias, and data exists on the role of the mitochondrial genome in the occurrence and development of HCM. Nevertheless, the data of morphologic study of ultrastructures of myocardium in HCM are underrepresented in the literature. Changes in the ultrastructural characteristics of myocardial mitochondria in HCM and their relationship with other structural and functional features of the tissue require clarification using extensive and phenotypically diverse material.

Aim. To study the ultrastructure of mitochondria of myocardial biopsy specimens of interventricular septum of a pilot group of patients with hypertrophic cardiomyopathy of diffuse-generalized phenotype.

Material and methods. The material of intraoperative biopsies of myocardium of the interventricular septum of 20 patients with the diagnosis “hypertrophic cardiomyopathy, diffuse-generalized phenotype” was studied by transmission electron microscopy (TEM). Myocardial samples from 2 patients operated for aortic valve stenosis were used as controls.

Results. Changes characteristic for HCM, such as hyperplasia of mitochondria, changes in their ultrastructure indicating increased functional activity, changes in the localization of mitochondria in the cell, signs of mitochondrial degradation, accumulation of lipofuscin pigment in cells were revealed.

Conclusion. The results of the study indicate relatively successful compensation of energy deficit in the myocardium of patients with HCM at this stage of ontogenesis. The obtained data allow us to analyze the pathogenesis of this disease and to predict the effectiveness of the process of postoperative rehabilitation of patients in terms of compensatory-adaptive reactions of a cell.

Keywords:hypertrophic cardiomyopathy; transmission electron microscopy; ultrastructural pathology; sarcomeric mutations; morphometry; mitochondria; hypoxia

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