Endocardial Fibroelastosis

Endokardiell fibroelastos

Svensk definition

Ett tillstånd kännetecknat av hypertrofi i vänstra kammarens vägg och omvandling av endokardiet till en tjock, fibrös och elastisk hinna, vilket ibland leder till minskad kammarkapacitet, men ofta till ökad.

Engelsk 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.

Svenska synonymer

Inga svenska synonymer finns.

Engelska synonymer

Endocardial Fibroelastoses Fibroelastoses, Endocardial Fibroelastosis, Endocardial Endomyocardial Fibroelastosis