Abstract
A case of dermatomyositis associated with recurrent syncope and complete heart block is described. Right bundle branch block and left anterior hemiblock with intact atrioventricular conduction then persisted over a five-year period. His bundle studies demonstrated a prolonged His-ventricle (H-V) interval. Postmortem examination confirmed the diagnosis of dermatomyositis as well as a fibrotic cardiomyopathy. The pathologic findings of marked fibrosis of both bundle branches, especially the right bundle branch and the anterior portion of the main left bundle branch, correlated well with the electrocardiographic and electrophysiologic findings.
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