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(Circulation. 2003;107:2519.)
© 2003 American Heart Association, Inc.

Images in Cardiovascular Medicine

Myocardial Fibrosis in Fabry Disease Demonstrated by Multislice Computed Tomography

Comparison With Biopsy Findings

Nobusada Funabashi, MD; Tetsuya Toyozaki, MD; Yasunori Matsumoto, MD; Masayori Yonezawa, MD; Noriyuki Yanagawa, RT; Katsuya Yoshida, MD; Issei Komuro, MD

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.

Correspondence 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. E-mail komuro-tky{at}umin.ac.jp

A54-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 -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.

View larger version (58K): [in this window] [in a new window]   Figure 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.

View larger version (92K): [in this window] [in a new window]   Figure 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.

Acknowledgments

This work was supported by Grant from Takeda Science Foundation.

Footnotes

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.

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Funabashi, N. Articles by Komuro, I. Search for Related Content PubMed PubMed Citation Articles by Funabashi, N. Articles by Komuro, I. Related Collections CT and MRI