Loperfido F, Fiorilli R, Santarelli P, Bellocci F, Zecchi P
Acta Cardiol. 1982;37(1):31-8.
A case of progressive systemic sclerosis with syncopal symptoms is reported. The presenting ECG pattern was that of an anterior myocardial infarction. The clinical history and the coronary angiography excluded significant coronary atherosclerotic heart disease. The ECG pattern evolved from the infarctual pattern associated with right bundle branch block to probably major degree of right bundle branch block associated with left posterior fascicular block. M-mode echocardiography, heart catheterization and angiographic studies did not reveal significant mechanical impairment of the left or right ventricle function. His bundle electrogram documented a markedly prolonged H-V interval, confirming an advanced impairment of distal conducting system. This case supports the suggestion that intraventricular conduction disorders in sclerodermal heart disease are not always related to diffuse myocardial involvement. The risk of sudden death justifies accurate electrophysiological evaluation in selected patients with sclerodermal cardiopathy.
报告了一例伴有晕厥症状的进行性系统性硬化症病例。最初的心电图表现为前壁心肌梗死。临床病史和冠状动脉造影排除了严重的冠状动脉粥样硬化性心脏病。心电图表现从与右束支传导阻滞相关的梗死图形演变为可能与左后分支阻滞相关的严重右束支传导阻滞。M型超声心动图、心导管检查和血管造影研究未发现左心室或右心室功能有明显的机械性损害。希氏束电图显示H-V间期明显延长，证实远端传导系统有严重损害。该病例支持以下观点：硬皮病性心脏病中的室内传导障碍并不总是与弥漫性心肌受累有关。猝死风险使得对部分硬皮病性心肌病患者进行准确的电生理评估成为必要。
