Infantile xanthomatous cardiomyopathy

Abstract

A 10-month-old female infant with Wolff-Parkinson-White syndrome and atrlal and ventricular tachyarrhythmias is described. She developed atrial fibrillation with a ventricular rate of 400 per minute. With antiarrhythmic drug therapy she survived 9 months. Autopsy showed typical features of infantile xanthomatous cardiomyopathy with vacuolated fatty cells replacing the normal myofibres. The Wolff-Parkinson-White syndrome has occurred in other cases of this rare cardiomyopathy which has a marked female predominance and occurs In children under the age of 2 years.