endocardial fibroelastosis

Endocardial fibroelastosis (EFE) is a rare cardiac condition which is classically described in the pediatric population (typically first two years). It is one of the causes in infants of unexplained heart failure.

Epidemiology

Associations

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

Pathology

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

Radiographic features

Ultrasound

Echocardiography may show:

• strongly echogenic myocardium
• ancillary features, e.g. fetal cardiomegaly and evidence of hydrops fetalis as a result of cardiac failure

MRI

Cardiac MRI might show:

• endocardial thickening
• apical filling defect
• atrial or ventricular thrombus formation
• diffuse endocardial late gadolinium enhancement 

Siehe auch:

• Hydrops fetalis
• hypoplastic left heart syndrome
• Mitralklappeninsuffizienz
• fetal cardiomegaly
• Barth-Syndrom

und weiter:

• fetal cardiomyopathy
• Endokard