Endocardial fibroelastosis

Description

Endocardial fibroelastosis is the formation of a marked fibro-elastic thickening of the subendocardium in one or both cardiac ventricles. A disorder of fetuses and infants, secondary causes include congenital left-sided obstructive cardiac lesions, metabolic disorders, autoimmune disease (anti-Ro/anti-La antibodies), and transplacental viral infection such as mumps. Primary endocardial fibroelastosis has been linked to recessive and x-linked inheritance, such as with Barth syndrome.

Index terms

Endocardial fibroelastosis
EFE - [endocardial fibroelastosis]
congenital endocardial fibroelastosis

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congenital valvular endocarditis (deprecated)
elastomyofibrosis
endomyocardial fibroelastosis
fetal endocarditis
fibroelastosis
fibroelastosis cordis
primary endocardial fibroelastosis
Congenital endocarditis
Congenital cardiomyopathy
Congenital familial cardiomyopathy
Congenital subendocardial sclerosis

Exclusions from above levels

BC42 – Myocarditis
01 – Certain infectious or parasitic diseases
05 – Endocrine, nutritional or metabolic diseases

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18 – Complications of pregnancy, childbirth and the puerperium
19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations and chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

BC43.0 – Dilated cardiomyopathy
BC43.1 – Hypertrophic cardiomyopathy
BC43.2 – Restrictive cardiomyopathy
BC43.4 – Cardiomyopathy due to drugs or other external agents
BC43.5 – Stress-induced cardiomyopathy
BC43.6 – Arrhythmogenic ventricular cardiomyopathy
BC43.7 – Diabetic cardiomyopathy
BC43.Y – Other specified cardiomyopathy
BC43.Z – Cardiomyopathy, unspecified

Foundation entity

Endocardial fibroelastosis – http://id.who.int/icd/entity/1971033419

Allowed postcoordination axes

hasManifestation

Part of: BC43: Cardiomyopathy › 11: Diseases of the circulatory system