A48-year-old woman was admitted for further cardiological exploration because echocardiography had revealed an atypical inner contour of the left ventricle and mild left ventricular dysfunction. Her major complaint was dyspnea on exertion. She had suffered from myocarditis 20 years earlier. Physical examination showed no abnormality; the ECG showed a right bundle-branch block.
To clarify the situation, an electron-beam CT examination was performed. It confirmed the echocardiographic findings but also revealed fatty tissue within the left and right ventricular walls, leading to the diagnosis of biventricular dysplasia (Figure 1⇓). Coronary artery disease was ruled out by cardiac catheterization. However, right and also left ventricular contrast angiograms showed typical regional wall motion abnormalities (Figures 2⇓ and 3⇓). Finally, the diagnosis was validated by endomyocardial biopsy showing fibrofatty infiltration of the right ventricular wall (Figures 4⇓ and 5⇓).
No malignant arrhythmias were observed in our patient during repeated ambulatory ECG monitoring or during exercise testing. In the past 2 years she has done well, and she is being seen regularly by a cardiologist. Examination of her relatives was negative, and we could find no hereditary involvement of the family.
Arrhythmogenic right ventricular dysplasia is characterized by progressive replacement of the myocardium by fibroadipose tissue and ventricular arrhythmias.1 Biventricular involvement in association with right ventricular dysplasia has also been described.2 MRI and electron beam CT can noninvasively identify fatty infiltration in the ventricular walls and lead to the correct diagnosis.3 4
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 and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
- Copyright © 2000 by American Heart Association
Fontaine G, Fontaliran F, Frank R. Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses. Circulation. 1998;97:1532–1535.
Tada H, Shimizu W, Ohe T, Hamada S, Kurita T, Aihara N, Kamakura S, Takamiya M, Shimomura K. Usefulness of electron-beam computed tomography in arrhythmogenic right ventricular dysplasia: relationship to electrophysiological abnormalities and left ventricular involvement. Circulation. 1996;94:437–444.