MR findings of endocardial fibroelastosis in children.
BACKGROUND
Endocardial fibroelastosis (EFE) is characterized by a diffuse white fibrous tissue lining the endocardium. The diagnosis is difficult to establish because clinical symptoms and electrocardiographic findings are nonspecific. Surgical resection of EFE requires the establishment of the diagnosis and delineation of the extent of the fibrotic changes.


OBJECTIVE
To describe the use of MRI in the assessment of EFE in children.


METHODS
Three children after surgery for aortic stenosis who were suspected of having EFE were evaluated by echocardiography and MRI. The MR evaluation consisted of black-blood, triple IR, bright-blood, perfusion and myocardial delayed-enhancement sequences. EFE was confirmed at surgery in all patients.


RESULTS
Echocardiograms demonstrated vigorous systolic function but substantial diastolic dysfunction of the left ventricle in all. Mild endocardial brightening of the anterior septum, anterior wall, or papillary muscles was present in two. No study was thought to be diagnostic of endocardial fibrosis. On MRI EFE manifested at the endocardial surface as a rim of hypointense signal in the perfusion sequences and as a rim of hyperintense signal in the myocardial delayed-enhancement sequences. The black-blood, triple IR, and bright-blood sequences were not diagnostic.


CONCLUSIONS
The diagnosis of EFE is difficult to establish by echocardiography. MRI using perfusion and myocardial delayed enhancement can be useful in establishing the diagnosis.
Stranzinger
Enno
E
Section of Pediatric Radiology, University of Michigan Health System, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0252, USA. enno@med.umich.edu
Ensing
Gregory J
GJ
Hernandez
Ramiro J
RJ
D001024:Aortic Valve Stenosis / Q000601:surgery; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D004695:Endocardial Fibroelastosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male