(Circulation. 2000;101:1479.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Biventricular Dysplasia
From the Department of Medicine, Division of Cardiology (N.W., P.L., F.M.F., W.K.); the Department of Radiology (R. Kern, R.R.); and the Department of Pathology, Laboratory of Neuropathology (R. Kleinert), University of Graz, Austria.
Correspondence to Norbert Watzinger, MD, University of Graz, Department of Medicine, Division of Cardiology, Auenbruggerplatz 15, A-8036 Graz, Austria. E-mail norbert.watzinger{at}kfunigraz.ac.at
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
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 and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
References
1.
Fontaine G, Fontaliran F, Frank R. Arrhythmogenic
right ventricular cardiomyopathies:
clinical forms and main differential diagnoses.
Circulation. 1998;97:1532–1535.
2. Pinamonti B, Sinagra G, Salvi A, Di Lenarda A, Morgera T, Silvestri F, Bussani R, Camerini F. Left ventricular involvement in right ventricular dysplasia. Am Heart J. 1992;123:711–724.[Medline] [Order article via Infotrieve]
3.
Blake LM, Scheinman MM, Higgins CB. MR features of
arrhythmogenic right ventricular dysplasia. Am J
Roentgenol. 1994;162:809–812.
4.
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.
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||