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Endocardial fibroelastosis

Dr Yuranga Weerakkody et al.

Endocardial fibroelastosis (EFE) is a rare cardiac condition which is classically described in the paediatric population (typically ~ 1^st two years). It is one of the causes for infants to present with unexplained heart failure.

Pathology

The condition results from increasing amounts of fibrous an elastic tissue affecting the endocardium as well as the sub endocardium. This then causes diffuse thickening with subsequent impairment of myocardial contractility.

Assocations

mitral valve abnormalities : e.g mitral regurgitation
hypoplastic left heart syndrome : along the wall of the hypoplastic left ventricle
Barth syndrome

Radiographic features

Ultrasound - echocardiography

may show a strongly echogenic myocardium ³
may also show ancillary features such as fetal cardiomegaly and evidence of hydrops fetalis as a result of cardiac failure.

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