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Endocardial Fibroelastosis

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Definition: A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.     
Examples Isolated Noncompaction of the Ventricular Myocardium
Other names Fibroelastosis, Endocardial; Fibroelastoses, Endocardial; Endocardial Fibroelastoses
 
SubstanceCAS Registry & nameCategoriesSource
Hydrocephalus, endocardial fibroelastosis, and cataracts  0   *Cataract *Endocardial Fibroelastosis *Hydrocephalus.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012

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