Myocardial Fibrosis in Fabry Disease Demonstrated by Multislice Computed Tomography
2003; Lippincott Williams & Wilkins; Volume: 107; Issue: 19 Linguagem: Inglês
10.1161/01.cir.0000062036.35852.01
ISSN1524-4539
AutoresNobusada Funabashi, Tetsuya Toyozaki, Yasunori Matsumoto, Masayori Yonezawa, Noriyuki Yanagawa, Katsuya Yoshida, Issei Komuro,
Tópico(s)Cardiomyopathy and Myosin Studies
ResumoHomeCirculationVol. 107, No. 19Myocardial Fibrosis in Fabry Disease Demonstrated by Multislice Computed Tomography Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBMyocardial Fibrosis in Fabry Disease Demonstrated by Multislice Computed TomographyComparison With Biopsy Findings Nobusada Funabashi, Tetsuya Toyozaki, Yasunori Matsumoto, Masayori Yonezawa, Noriyuki Yanagawa, Katsuya Yoshida and Issei Komuro Nobusada FunabashiNobusada Funabashi From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. , Tetsuya ToyozakiTetsuya Toyozaki From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. , Yasunori MatsumotoYasunori Matsumoto From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. , Masayori YonezawaMasayori Yonezawa From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. , Noriyuki YanagawaNoriyuki Yanagawa From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. , Katsuya YoshidaKatsuya Yoshida From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. and Issei KomuroIssei Komuro From the Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine (N.F., T.T., Y.M., M.Y., K.Y., I.K.); and the Department of Radiology, Chiba University Hospital (N.Y.), Chiba, Japan. Originally published20 May 2003https://doi.org/10.1161/01.CIR.0000062036.35852.01Circulation. 2003;107:2519–2520A 54-year-old man presented with dyspnea on effort. Echocardiogram revealed reduced apical wall motion of the left ventricle (LV) with extreme hypertrophy of the interventricular septum (IVS). Conventional coronary angiogram showed normal coronary arteries. Endomyocardial-biopsy specimens obtained from the IVS revealed extensive vacuolization of cardiac myocytes and mild fibrosis on light microscopy, and typical lysosomal inclusions with a concentric lamellar configuration were seen with electron micros-copy (Figure 1). With these findings and low plasma α-galactosidase activity, he was diagnosed as having Fabry disease. To evaluate the characteristics of the LV, ECG-gated enhanced multislice computed tomography (CT) (Light Speed Ultra, General Electric) was performed with a 1.25-mm slice thickness, helical pitch 3.25. After intravenous injection of 100 mL of iodinated contrast material (350 mgI/mL), CT scanning was performed with retrospective ECG-gated reconstruction at 30 seconds and 8 minutes after injection. In the axial source images, extreme hypertrophy of the IVS and the posterior wall of the LV compared with the apical and lateral walls of the LV could be observed (Figure 2). The apical and lateral portions of the LV revealed lower CT intensity than the IVS in the early phase (arrows), and in the late phase they were abnormally enhanced compared with the IVS, suggesting fibrotic changes in the apical and lateral myocardium. Therefore, we concluded that despite the IVS biopsy results, more fibrotic changes occurred in the apical and lateral portions of the LV rather than in the IVS. Download figureDownload PowerPointFigure 1. Histological findings of endomyocardial-biopsy specimens. A, Photomicrograph shows sarcoplasmic vacuolization of cardiac myocytes and fibrosis (hematoxylin and eosin staining). B, Electron micrograph shows typical lysosomal inclusions with a concentric lamellar configuration.Download figureDownload PowerPointFigure 2. Axial source images of enhanced multislice computed tomography acquired 30 seconds (early phase) and 8 minutes (late phase) after the injection of the contrast material. Images show extreme hypertrophy of the interventricular septum (IVS) and posterior wall compared with the apical and lateral walls of the left ventricle (LV). The apical and lateral portions revealed lower computed tomography intensity than the IVS in the early phase (arrows). Conversely, in the late phase, the apical and lateral portions of the LV (arrows) were abnormally enhanced compared with the extremely hypertrophic IVS, suggesting more fibrotic changes in the apical and lateral myocardium. RV indicates right ventricle.The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.This work was supported by Grant from Takeda Science Foundation.FootnotesCorrespondence to Issei Komuro, MD, Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba City, Chiba 260-8670, Japan. 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