Endocardial fibroelastosis

Orpha code: 2022OMIM code: 226000

Definition

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.

Disease data
Classification

Disease

Synonyms
Endomyocardial fibroelastosis
ORPHA code
2022
OMIM code
226000
ICD10 code
I42.4
ICD11 code
BC43.3

No additional description.

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