Idiopathic Cardiomyopathy in Children

This report describes 27 cases of a clinical cardiomyopathic syndrome in clinically well children. Physical findings regularly observed include an abnormal left ventricular impulse, a prominent X and Y descent in the jugular venous pulse, and a characteristic, acquired, systolic murmur. These are associated with the laboratory findings of an enlarged left ventricle by electrocardiogram and x-ray. This syndrome may be the earliest manifestation of cardiomyopathic states, such as idiopathic myocardial hypertrophy or hypertrophic outflow tract obstruction, which usually appear in the third decade of life and beyond. The frequency of cases found suggests that the latter diseases, which are relatively rare, are possibly only the more malignant representatives of a broad spectrum of cardiomyopathic states.