Evidence has been presented which indicates that endocardial fibrosis is a relatively common cause of death from heart disease in infancy and that a presumptive diagnosis of this condition can often be made during life. The salient features are: age under one year, marked dyspnea without persistent cyanosis, absence of a loud cardiac murmur on repeated examinations, enlargement of the heart, presence of femoral pulsations, and electrocardiographic evidence of left ventricular hypertrophy in the precordial leads. The chief conditions to be considered in the differential diagnosis are: aberrant coronary artery, interstitial myocarditis, and glycogen storage disease of the heart, all of which are much less frequent in the autopsy data. Four cases are presented in detail. In two of these the diagnosis was correctly made before death.
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