This study was supported in part by a grant from the George M. Eisenberg Foundation and by Grant UO1-HL65594 from the National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia
Article first published online: 11 NOV 2008
© 2009 Wiley Periodicals, Inc.
Journal of Cardiovascular Electrophysiology
Volume 20, Issue 5, pages 473–476, May 2009
How to Cite
VASAIWALA, S. C., FINN, C., DELPRIORE, J., LEYA, F., GAGERMEIER, J., AKAR, J. G., SANTUCCI, P., DAJANI, K., BOVA, D., PICKEN, M. M., BASSO, C., MARCUS, F. and WILBER, D. J. (2009), Prospective Study of Cardiac Sarcoid Mimicking Arrhythmogenic Right Ventricular Dysplasia. Journal of Cardiovascular Electrophysiology, 20: 473–476. doi: 10.1111/j.1540-8167.2008.01351.x
- Issue published online: 21 APR 2009
- Article first published online: 11 NOV 2008
- Manuscript received 11 June 2008; Revised manuscript received 16 September 2008; Accepted for publication 19 September 2008.
- arrhythmogenic right ventricular dysplasia;
- cardiac sarcoid;
- myocardial biopsy;
- left ventricular dysfunction;
- heart block
Introduction: Case studies indicate that cardiac sarcoid may mimic the clinical presentation of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, the incidence and clinical predictors to diagnose cardiac sarcoid in patients who meet International Task Force criteria for ARVD/C are unknown.
Methods and Results: Patients referred for evaluation of left bundle branch block (LBBB)-type ventricular arrhythmia and suspected ARVD/C were prospectively evaluated by a standardized protocol including right ventricle (RV) cineangiography-guided myocardial biopsy. Sixteen patients had definite ARVD/C and four had probable ARVD/C. Three patients were found to have noncaseating granulomas on biopsy consistent with sarcoid. Age, systemic symptoms, findings on chest X-ray or magnetic resonance imaging (MRI), type of ventricular arrhythmia, RV function, ECG abnormalities, and the presence or duration of late potentials did not discriminate between sarcoid and ARVD/C. Left ventricular dysfunction (ejection fraction <50%) was present in 3/3 patients with cardiac sarcoid, but only 2/17 remaining patients with definite or probable ARVD/C (P = 0.01).
Conclusions: In this prospective study of consecutive patients with suspected ARVD/C evaluated by a standard protocol including biopsy, the incidence of cardiac sarcoid was surprisingly high (15%). Clinical features, with the exception of left ventricular dysfunction and histological findings, did not discriminate between the two entities.