FAMILIAL CARDIOMYOPATHY

Abstract
A group of families, the descendants of one couple through four generations, is described In these families 9 cases of familial cardiomyopathy were discovered, and 6 other probable cases. Five of these 15 have died suddenly, while two lived one day and a third was stillborn. The remaining seven are still alive, and four with no symptoms. The environment of these families is discussed and also the clinical, radiological, electrocardiographic, and pathological findings. The mode of inheritance appears due to a Mendalian dominant gene with incomplete penetrances. A metabolic defect in cardiac muscle has been demonstrated in two of the cases coming to necropsy. This consisted of finding a non-meta-chromatic neutral polysaccharide in the cardiac muscle fibres. The etiology of this condition is reviewed, and it is suggested that the cause of this condition is an inherited metabolic defect in cardiac muscle.