CONGENITAL ENDOCARDIAL FIBRO-ELASTOSIS

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Abstract
Seventeen cases of congenital endocardial fibro-elastosis without associated cardiac malformations or other known cause have been reported. Seventy-nine similar cases have been collected from the literature. Evidence is presented that these cases represent a disease entity with the following characteristic features: a. The disease is familial. b. Nonspecific symptoms begin in the first 6 months of life in the majority of cases, consisting of failure to gain and cough, over a few days or weeks. A sudden attack of labored breathing and tachycardia with vomiting or abdominal pain may lead to death within 1 or 2 days or to recovery followed by repetition of similar attacks. Death within a day of onset of an attack occurs in half of the cases. c. Bradycardia before birth and congenital heart block occurred in two cases. d. The heart is grossly enlarged mainly because of dilatation and hypertrophy of the left ventricle. e. The endocardial fibro-elastosis increases with age and is most marked in the left ventricle. f. Non-cardiac malformations are rarely present. A hypothesis is presented that the disease is based on a familial metabolic defect leading to myocardial weakness and that the endocardial changes are secondary.

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