title: Electron beam computed tomography appearance of endocardial fibroelastosis EBCT appearance of endocardial fibroelastosis.
abstract: Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan, ROC.
authors.forename: I-Jen
authors.initials: IJ
authors.lastname: Wang
authors.forename: Shyh-Jye
authors.initials: SJ
authors.lastname: Chen
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.forename: Chung-I
authors.initials: CI
authors.lastname: Chang
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D000328:Adult; D002648:Child; D002675:Child, Preschool; D017023:Coronary Angiography; D003330:Coronary Vessel Anomalies / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D004452:Echocardiography; D004695:Endocardial Fibroelastosis / Q000175:diagnosis* / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D014057:Tomography, X-Ray Computed / Q000379:methods*