Ridolfi R L, Bulkley B H, Hutchins G M
Am J Med. 1976 Sep;61(3):361-6. doi: 10.1016/0002-9343(76)90373-9.
Progressive systemic sclerosis may be associated with focal myocardial fibrosis. Electrocardiographic abnormalities including conduction block are common in progressive systemic sclerosis but whether they are due to direct destruction of the specialized conduction tissue of the heart is uncertain. The conduction systems of 35 patients with progressive systemic sclerosis were studied. Of these 35 patients, 17 (50 per cent) had myocardial fibrosis of the type seen in progressive systemic sclerosis. In 10 of the 17, it was severe. Sinus node fibrosis was present in 13 patients and was nearly as frequent in those with as in those without the progressive systemic sclerosis myocardial lesion. Overlying pericarditis may have contributed to the fibrotic changes within the sinoatrial nodes in seven of the 13 patients. The atrioventricular node and main His bundles were normal. However, fibrotic changes were found in the proximal bundle systems in six patients. In three of the six, severe myocardial progressive systemic sclerosis was present, two had focal fibrous atrophy of the left bundle, and one had complete interruption of the right bundle. In only the latter patient was this reflected in the electrocardiogram which showed a right bundle branch block. Three patients without progressive systemic sclerosis myocardial lesions also had fibrous atrophy of a portion of the proximal left bundle branch, and in one the electrocardiogram showed an isolated left anterior hemiblock. Thus, morphologic abnormalities within the conduction system in our patients are difficult to attribute to progressive systemic sclerosis per se. Furthermore, although conduction abnormalities were more frequent in patients with myocardial disease, specific conduction system disease was not the cause in most patients. As has been noted in ischemic heart disease, the conduction system appears to be relatively spared from the myocardial changes of progressive systemic sclerosis, and the high incidence of conduction disturbances in this condition may be a consequence, rather, of damage to working myocardium.
进行性系统性硬化症可能与局灶性心肌纤维化有关。心电图异常包括传导阻滞在进行性系统性硬化症中很常见，但它们是否是由于心脏特殊传导组织的直接破坏尚不确定。对35例进行性系统性硬化症患者的传导系统进行了研究。在这35例患者中，17例（50%）有进行性系统性硬化症中所见类型的心肌纤维化。在这17例中的10例，纤维化严重。13例患者存在窦房结纤维化，在有和没有进行性系统性硬化症心肌病变的患者中出现频率相近。13例中的7例，覆盖性心包炎可能促成了窦房结内的纤维化改变。房室结和希氏束主干正常。然而，在6例患者的近端束支系统中发现了纤维化改变。6例中的3例存在严重的心肌进行性系统性硬化症，2例有左束支局灶性纤维萎缩，1例右束支完全中断。仅在最后1例患者的心电图上有反映，显示右束支传导阻滞。3例没有进行性系统性硬化症心肌病变的患者也有近端左束支部分纤维萎缩，其中1例心电图显示孤立性左前分支阻滞。因此，我们患者传导系统内的形态学异常难以单纯归因于进行性系统性硬化症。此外，尽管传导异常在心肌疾病患者中更常见，但大多数患者的病因并非特定的传导系统疾病。正如在缺血性心脏病中所指出的，传导系统似乎相对未受进行性系统性硬化症心肌改变的影响，这种情况下传导障碍的高发生率可能是工作心肌受损的结果，而非传导系统本身病变所致。
