PEDIATRICS Vol. 48 No. 2 August 1971, pp. 312-315
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Infantile Xanthomatous Cardiomyopathy

H. Edward MacMahon M.D., M.R.C.P.1

1 Department of Pathology, Tufts University School of Medicine and Dental Medicine, 136 Harrison Avenue, Boston, Massachusetts 02111

This report deals with a most unusual disease of the heart that was characterized clinically by irreversible atrial tachycardia and recurrent ventricular fibrillation. This led to the death of a 13-month-old female infant. Anatomically, xanthomatous clusters of closely packed lipid-containing cells displaced segments of the myocardium. Neither the etiology nor pathogenesis of this disease is known. This is the seventh such case to be recorded.