Endocardial fibroelastosis
- Endocardial fibroelastosis
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OMIM = 226000
OMIM_mult = OMIM2|305300
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Endocardial fibroelastosis (EFE) is a rare congenital heart disorder that is characterized by a thickening within the muscular lining of the heart chambers (the endocardium) due to an increase in the amount of supporting connective tissue and elastic fibers. It is an uncommon cause of unexplained heart failure in infants and children. It is one component of HEC syndrome.
EFE has two distinct genetic forms, each having a different mode of inheritance. An x-linked recessive form, [OMIM|226000] and an autosomal recessive form [OMIM|305300] have both been observed.
The term was introduced by Weinberg and Himmelfarb in 1943. [Weinberg T, Himmelfarb AJ (1943). "Endocardial fibroelastosis". Bull Johns Hopkins Hosp. 72: 299.]
References
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2010.
Look at other dictionaries:
endocardial fibroelastosis — n a condition usu. associated with congestive heart failure and enlargement of the heart that is characterized by conversion of the endocardium to fibroelastic tissue * * * diffuse patchy thickening of the mural endocardium, particularly in the… … Medical dictionary
primary endocardial fibroelastosis — a congenital form of endocardial fibroelastosis, manifest in infancy and occurring unassociated with other cardiac defects … Medical dictionary
fibroelastosis — Excessive proliferation of collagenous and elastic fibrous tissue. endocardial f., endomyocardial f. 1. a congenital condition characterized by thickening of the left ventricular wall endocardium (c … Medical dictionary
fibroelastosis — n. overgrowth or disturbed growth of the yellow (elastic) fibres in connective tissue, especially endocardial fibroelastosis, overgrowth and thickening of the inner layer of the heart s left ventricle … The new mediacal dictionary
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