Does Endocardial Fibroelastosis in Children Have Characteristic MRI Findings?

The objective of a recent study was to describe findings on MRI that correlate with a diagnosis of Endocardial Fibroelastosis (EFE) in children, and to compare its utility with that of echocardiographic examination.

The study has concluded that MRI findings that correlate with endocardial fibroelastosis in children include a hypointense layer at the endocardial surface, which becomes bright on delayed-enhancement images.

Participants/Methodology
Three children who were referred to radiology for a suspected diagnosis of EFE were identified. The children had all undergone cardiac catheterization and angioplasty, but had persistent left ventricular (LV) dysfunction and pulmonary hypertension.

Multiple echocardiographic evaluations had been performed as part of their clinical care. All three children underwent a cardiac MRI under general anesthesia in a 1.5-T magnet. Perfusion and myocardial delayed-enhancement sequences were also performed.

Results of the Study
Echocardiogram in all three children revealed hyperdynamic LV systolic function with variable ejection fractions. All children had evidence of LV diastolic dysfunction with substantial left atrial enlargement. Echocardiographic assessment of the LV endocardium revealed mild brightening of the anterior septum in two patients, with the third patient having minimal brightening.

On cardiac MRI, early perfusion sequences showed a hypointense inner layer of endocardium in all three children. This same layer showed hyper-enhancement on delayed enhancement sequences. The best signal contrast between the area of endocardial fibroelastosis and adjacent myocardium was noted after a 20- to 30-minute delay after gadolinium administration. All three children had surgical confirmation and removal of EFE.

Conclusions
Children with EFE present clinically with signs of congestive heart failure and elevated pulmonary pressure. Although the clinical picture may be suggestive, echocardiography is not very specific when attempting to evaluate patients with this presumptive diagnosis. Early demonstration of the utility of MRI in assessing this condition is trending positively.

Reviewer’s Comments
The MRI characteristics presented in this study would be expected considering the histologic characteristics of EFE. The enhancement dynamics are similar to well-documented findings associated with myocardial infarction, which has a component of fibrosis as well. Although a rare diagnosis, in centers that deal with pediatric cardiac disease, MRI is showing its utility over conventional echocardiography in some conditions.

Author: Basil Hubbi, MD).

Reference
Stranzinger E, Ensing GJ, Hernandez RJ. MR Findings of Endocardial Fibroelastosis in Children. Pediatr Radiol; 2008; 38 (March): 292-296: