Endocardial Fibroelastosis

Endocardial fibroelastosis is a rare condition that results in diffuse thickening of the ventricular endocardium and presents as unexplained heart failure in infants and children.

It can be divided into primary and secondary forms. Primary endocardial fibroelastosis is not associated with any significant structural cardiac abnormality, while secondary endocardial fibroelastosis is associated with congenital cardiac abnormalities, most commonly hypoplastic left heart syndrome and aortic stenosis.

Echocardiography may show echogenic endocardial surface and a spherical shape to the left ventricle, but this appearance has a weak correlation with endocardial fibroelastosis on histology. Perfusion MRI shows a hypointense layer at the endocardial surface that corresponds to an area of delayed enhancement.

References

• Stranzinger E, Ensing GJ, Hernandez RJ. MR findings of endocardial fibroelastosis in children. Pediatr Radiol. 2008 Mar;38(3):292-6.
• Tworetzky W, del Nido PJ, Powell AJ, Marshall AC, Lock JE, Geva T. Usefulness of magnetic resonance imaging of left ventricular endocardial fibroelastosis in infants after fetal intervention for aortic valve stenosis. Am J Cardiol. 2005 Dec 1;96(11):1568-70.