Becker A E, Caruso G
Br Heart J. 1982 Jun;47(6):527-38. doi: 10.1136/hrt.47.6.527.
Myocardial disarray or disorganisation is at present a contentious topic, not least because its value as a clinical marker for hypertrophic cardiomyopathy has changed considerably over the years. Initially observed as one of the features of asymmetric septal hypertrophy, disarray has since been promoted as its pathognomonic histological feature, regarded by some observers as the morphological manifestation of a genetically transmitted myocardial defect. Recently, however, it has become evident that myocardial disarray is not limited to hypertrophic cardiomyopathy, but is encountered in hearts with both congenital and acquired conditions, and is also observed in normal hearts. The specificity of disarray for hypertrophic cardiomyopathy is thus seriously questioned. Latterly, it has been suggested that disarray, judged from through-and-through sections of the ventricular midseptum is a highly specific and sensitive marker of hypertrophic cardiomyopathy when considered in quantitative rather than qualitative fashion. The present study sets out to answer the question whether disarray could be the histological expression of the normal but intricate fibre architecture of the heart, a consideration also initiated by debatable definitions of normality and abnormality of myocardial histology. Gross fibre dissections in five normal hearts showed that many sites occurred in which disarray was a natural phenomenon. In five more hearts it was found that the plane of section of a tissue block might profoundly influence the histology. In fact, tissue cubicles sampled from different faces showed a change in histology in the vast majority. Thus the diagnostic significance of myocardial disarray as a marker of hypertrophic cardiomyopathy in the clinical setting almost vanishes; a change in orientation of a tissue section may actually turn "normality" into "disarray".
心肌排列紊乱目前是一个有争议的话题，尤其是因为多年来它作为肥厚型心肌病临床标志物的价值发生了很大变化。最初被观察为不对称性室间隔肥厚的特征之一，此后排列紊乱被提升为其特征性组织学特征，一些观察者认为这是遗传传递的心肌缺陷的形态学表现。然而，最近已经很明显，心肌排列紊乱不仅限于肥厚型心肌病，在先天性和后天性心脏病中也会出现，在正常心脏中也有观察到。因此，排列紊乱对肥厚型心肌病的特异性受到严重质疑。最近有人提出，从室间隔中部的全层切片判断，排列紊乱在以定量而非定性方式考虑时，是肥厚型心肌病的一种高度特异性和敏感性的标志物。本研究旨在回答排列紊乱是否可能是心脏正常但复杂的纤维结构的组织学表现这一问题，这一考虑也是由心肌组织学正常与异常的有争议定义引发的。对五个正常心脏进行大体纤维解剖显示，许多部位出现排列紊乱是一种自然现象。在另外五个心脏中发现，组织块的切片平面可能会对组织学产生深远影响。事实上，从不同面取样的组织小块在绝大多数情况下都显示出组织学变化。因此，在临床环境中，心肌排列紊乱作为肥厚型心肌病标志物的诊断意义几乎消失；组织切片方向的改变实际上可能会将“正常”转变为“排列紊乱”。
