Sukhacheva T V, Serov R A, Bockeria L A
A. N. Bakulev Center for Cardiovascular Surgery, Ministry of Health of the Russian Federation, Moscow, Russia.
Petrovsky National Research Center of Surgery, Avtsyn Research Institute of Human Morphology, Moscow, Russia.
Bull Exp Biol Med. 2024 Nov;178(1):130-138. doi: 10.1007/s10517-024-06295-6. Epub 2024 Nov 23.
The functioning of the ventricular myocardium in hypertrophic cardiomyopathy (HCM) under high hemodynamic load leads to depletion of its resources and is associated with the risk of developing a dilated stage. In patients with HCM, the cardiomyocytes of the interventricular septum are hypertrophied, the proportion of cardiomyocytes in which myofibrils constitute less than 50% of the sarcoplasm volume increases with increasing the cardiomyocyte length and correlates with echocardiographic signs of left ventricular obstruction. These cardiomyocytes are characterized by ultrastructural signs of synthetic activity. In the myocardium of patients with HCM, single cardiomyocytes with "critical" loss of myofibrils and nonspecific degenerative changes corresponding to the picture of irreversible changes in the cardiomyocyte ultrastructure were revealed. The presence of cardiomyocytes with this ultrastructural phenotype is an early marker of exhaustion of the compensatory capabilities of the myocardium in HCM.
肥厚型心肌病（HCM）患者的心室心肌在高血流动力学负荷下的功能会导致其资源耗尽，并与发展为扩张期的风险相关。在HCM患者中，室间隔的心肌细胞肥大，随着心肌细胞长度增加，肌原纤维占肌浆体积不到50%的心肌细胞比例增加，且与左心室梗阻的超声心动图征象相关。这些心肌细胞具有合成活性的超微结构特征。在HCM患者的心肌中，发现了单个具有“临界”肌原纤维丧失和对应于心肌细胞超微结构不可逆变化图像的非特异性退行性改变的心肌细胞。具有这种超微结构表型的心肌细胞的存在是HCM中心肌代偿能力耗尽的早期标志物。
