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October 1968

Myocardial Disease: A Case Associated With Hyperpigmentation and Multifocal Vascular Sclerosis

Author Affiliations

From the departments of pediatrics (Drs. Arcasoy and Guntheroth) and pathology (Dr. Smuckler), University of Washington School of Medicine, Seattle. Doctor Arcasoy is now with the Department of Cardiology, Hacettepe University School of Medicine, Ankara, Turkey.

Am J Dis Child. 1968;116(4):434-438. doi:10.1001/archpedi.1968.02100020438018

IN THE past decade, increasing interest has been demonstrated in cardiac enlargement which cannot be ascribed to common etiologic mechanisms such as hypertension, coronary arteriosclerosis, pulmonary disease, rheumatic fever, and congenital defects of the heart.1,2

Nadas et al3 proposed that the clinical picture of cardiomegaly and congestive failure without murmurs be called primary myocardial disease. They included the specific pathologic entities of endocardial fibroelastosis, idiopathic myocarditis, glycogen storage disease of the heart, and medial necrosis of the coronary arteries. Other known causes of myocardial disease are polyarteritis, lupus erythematosis, nutritional disorders, and idiopathic muscular hypertrophy with or without outflow obstruction and a syndrome called myocardosis.

Levy and his co-workers1,4 characterized myocardosis as consisting of cardiac hypertrophy, scarring of the myocardium and focal myocardial necrosis of unknown origin. Rapidly progressive heart failure associated with disturbances in rhythm and complicated by pulmonary and systemic embolization were recognized as common

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