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 diag...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric radiology 2008-03, Vol.38 (3), p.292-296
Main Authors: Stranzinger, Enno, Ensing, Gregory J., Hernandez, Ramiro J.
Format: Article
Language:English
Subjects:
Aortic Valve Stenosis - surgery
Child, Preschool
Echocardiography, Doppler
Endocardial Fibroelastosis - diagnosis
Endocardial Fibroelastosis - diagnostic imaging
Endocardial Fibroelastosis - surgery
Female
Humans
Imaging
Infant
Infant, Newborn
Magnetic Resonance Imaging - methods
Male
Medicine
Medicine & Public Health
Neuroradiology
Nuclear Medicine
Oncology
Original Article
Pediatrics
Radiology
Ultrasound
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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. Materials and 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. Conclusion The diagnosis of EFE is difficult to establish by echocardiography. MRI using perfusion and myocardial delayed enhancement can be useful in establishing the diagnosis.
ISSN:0301-0449
1432-1998
DOI:10.1007/s00247-007-0707-7