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Descriptor English: Endocardial Fibroelastosis
Descriptor Spanish: Fibroelastosis Endocárdica
Descriptor fibroelastosis endocárdica
Scope note: Afección caracterizada por engrosamiento del ENDOCARDIO debida a proliferación de tejido fibroso y elástico, generalmente en el ventrículo izquierdo que da lugar a deterioro de la función cardíaca (MIOCARDIOPATÍA RESTRICTIVA). Se observa más frecuentemente en niños jóvenes y rara vez en adultos. Se asocia con frecuencia con anomalías cardíacas congénitas (CARDIOPATÍAS CONGÉNITAS), INFECCIÓN o mutación génica. Existe una forma de fibroelastosis endocárdica familiar, autosómica dominante, que resulta de una alteración en la proteína tafazina, codificada por el gen TAZ.
Descriptor Portuguese: Fibroelastose Endocárdica
Descriptor French: Fibroélastose endocardique
Entry term(s): Endocardial Fibroelastoses
Endomyocardial Fibroelastosis
Fibroelastoses, Endocardial
Fibroelastosis, Endocardial
Tree number(s): C14.280.238.281
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D004695
Scope note: 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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
DeCS ID: 4776
Unique ID: D004695
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1966/01/01
Date of Entry: 1999/01/01
Revision Date: 2012/07/03
Endocardial Fibroelastosis - Preferred
Concept UI M0007391
Scope note 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.
Preferred term Endocardial Fibroelastosis
Entry term(s) Endocardial Fibroelastoses
Endomyocardial Fibroelastosis
Fibroelastoses, Endocardial
Fibroelastosis, Endocardial



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