In this family with congestive cardiomyopathy the lesion, a characteristic interstitial fibrosis with a high elastic tissue content, was defined at autopsy in 3 cases, and 3 further members may have been affected.The cases defined at autopsy all had longstanding arrhythmia and had suffered a cerebrovascular accident, probably secondary to endocardial fibroelastosis. The possibility that the myocardial cell changes may have been secondary to those in the interstitium is discussed. Ultrastructural Z band changes were demonstrated 5½ and 26 h postmortem.
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