Ferrans V J, Rodríguez E R
Pathology Branch, National Heart, Lung and Blood Institute, Bethesda, Maryland.
Z Kardiol. 1987;76 Suppl 3:20-5.
A review is presented of the mechanisms that mediate the increase in cardiac mass that occurs in patients with hypertrophic cardiomyopathy. This increase in mass is mediated by an increase in the total mass of the myocytes and the total mass of interstitial fibrous connective tissue. The increase in myocyte mass is the most important of these two components. However, it is not clear at the present time whether this increase is mediated not only by an increase in the size of the cells (hypertrophy), but also by an increase in the numbers of myocytes (hyperplasia) or by a combination of these two factors. Hyperplasia normally occurs during the prenatal phase of cardiac development and stops soon after birth, at which time hypertrophy becomes the main mechanism by which cardiac mass increases. Under certain circumstances, the ability of cardiac myocytes to synthesize DNA and undergo mitotic division can be restored; however, it is uncertain to what extent this results in complete cell division or only in either polyploidy or bi- or multinucleation. It is proposed that in hypertrophic cardiomyopathy, increased hyperplasia of cardiac myocytes occurs early in life and leads to permanently disturbed patterns of cardiac gross anatomy; it is then followed by a phase of progressive hypertrophy after the switch from hyperplastic to hypertrophic growth. It is possible that hyperplasia continues to occur after the usual time of this switch. It is also proposed that the increased number of layers of myocytes in the ventricular septum of patients with hypertrophic cardiomyopathy is a consequence of exaggerated hyperplasia during development.
本文综述了肥厚型心肌病患者心肌质量增加的介导机制。这种质量增加是由心肌细胞总质量和间质纤维结缔组织总质量的增加介导的。心肌细胞质量的增加是这两个组成部分中最重要的。然而,目前尚不清楚这种增加是否不仅由细胞大小的增加(肥大)介导,还由心肌细胞数量的增加(增生)或这两个因素的组合介导。增生通常发生在心脏发育的产前阶段,出生后不久就停止,此时肥大成为心脏质量增加的主要机制。在某些情况下,心肌细胞合成DNA和进行有丝分裂的能力可以恢复;然而,不确定这在多大程度上导致完全细胞分裂,还是仅导致多倍体或双核或多核。有人提出,在肥厚型心肌病中,心肌细胞增生增加在生命早期就会发生,并导致心脏大体解剖结构的永久性紊乱;随后在从增生性生长转变为肥大性生长后进入进行性肥大阶段。在这种转变的通常时间之后,增生可能继续发生。还有人提出,肥厚型心肌病患者室间隔中心肌细胞层数的增加是发育过程中过度增生的结果。