Disease or Syndrome
endocardial fibroelastosis
Subclass of:
Cardiomyopathies
Definitions related to endocardial fibroelastosis:
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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.NLM Medical Subject HeadingsU.S. National Library of Medicine, 2021
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A rare disorder characterized by diffuse thickening of the endocardium. It presents with unexplained heart failure.NCI ThesaurusU.S. National Cancer Institute, 2021
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Condition characterized by hypertrophy of the wall of the left ventricle and conversion of the endocardium into a thick fibroelastic coat, with the capacity of the ventricle sometimes reduced, but often increased.CRISP ThesaurusNational Institutes of Health, 2006
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Diffuse thickening of the ventricular endocardium and by associated myocardial dysfunction [PMID:23109776]Human Phenotype Ontology (HPO)The Human Phenotype Ontology Project, 2021
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Endomyocardial fibroelastosis is a cause of unexplained childhood cardiac insufficiency. It results from diffuse thickening of the endocardium leading to dilated myocardiopathy in the majority of cases and restrictive myocardiopathy in rare cases. It may occur as a primary disorder or may be secondary to another cardiac malformation, notably aortic stenosis or atresia.OrphaNetINSERM, 2021
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The term endocardial fibroelastosis (EFE) was introduced by Weinberg and Himmelfarb in 1943. Endocardial fibroelastosis refers to a pronounced, diffuse thickening of the ventricular endocardium and presents as unexplained heart failure in infants and children.WebMD, 2019
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Endomyocardial fibroelastosis is a cause of unexplained childhood cardiac insufficiency. It results from diffuse thickening of the endocardium leading to dilated myocardiopathy in the majority of cases and restrictive myocardiopathy in rare cases. It may occur as a primary disorder or may be secondary to another cardiac malformation,...National Center for Advancing Translational Sciences
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