Abstract
Purpose
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive cardiomyopathy characterized by myocardial atrophy and fibro-fatty replacement of the right ventricle (RV) and ventricular tachyarrhythmias in young patients. Our aim was to evaluate clinical course and electronic parameters in patients with implantable cardioverter-defibrillator (ICD) and ARVC, during long-term follow-up.
Methods and results
We report on 12 patients with ARVC (mean age 40 ± 13 years) who were treated with ICD implantation in our center. Although several RV sites were tested for proper lead positions, the amplitude of R-wave at implantation was quite low (7.4 ± 3.0 mV). After a mean follow-up of 91 ± 28 months, R-wave amplitude significantly decreased to a mean value of 5.4 ± 2.5 mV (p = 0.03). We also found a noticeable, nearly significant increase in pacing threshold (p = 0.052) and a moderate increase in defibrillation impedance (p = 0.07). Six patients (46 %) experienced at least one appropriate ICD therapy; three patients (23 %) experienced inappropriate ICD shocks secondary to the supraventricular tachycardia, T-wave oversensing, and electromagnetic interference.
Conclusions
ICD in patients with ARVC has been demonstrated to be feasible and safe. In our case series, we found low R-wave amplitudes at implantation and a significant R-wave decrease during follow-up; a considerable and nearly significant increase in pacing threshold was also observed. These findings may be related to the progressive fibro-fatty replacement of RV myocardium. Multiple sites should be tested in the right ventricle if sensing or pacing values are not optimal, and all the electronic parameters should be carefully monitored throughout the entire follow-up.
Similar content being viewed by others
References
Marcus, F. I., Fontaine, G. H., Guiraudon, G., Frank, R., Laurenceau, J. L., Malergue, C., et al. (1982). Right ventricular dysplasia: a report of 24 cases. Circulation, 65, 384–398.
Thiene, G., Nava, A., Corrado, D., Rossi, L., & Pennelli, N. (1988). Right ventricular cardiomyopathy and sudden death in young people. The New England Journal of Medicine, 318, 129–133.
Fontaine, G., Fontaliran, F., & Frank, R. (1998). Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnosis. Circulation, 97, 1532–1535.
Blomström-Lundqvist, C., Sabel, K. G., & Olsson, S. B. (1987). A long-term follow-up of 15 patients with arrhythmogenic right ventricular dysplasia. European Heart Journal, 58, 477–488.
Basso, C., Corrado, D., Marcus, F. I., Nava, A., & Thiene, G. (2009). Arrhythmogenic right ventricular cardiomyopathy. Lancet, 373, 1289–1300.
Breithardt, G., Wichter, T., Haverkamp, W., Borggrefe, M., Block, M., Hammel, D., et al. (1994). Implantable cardioverter defibrillator therapy in patients with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, or no structural heart disease. American Heart Journal, 127, 1151–1158.
Link, M. S., Wang, P. J., Haugh, C. J., Homoud, M. K., Foote, C. B., Costeas, X. B., et al. (1997). Arrhythmogenic right ventricular dysplasia: clinical results with implantable cardioverter-defibrillators. The Journal of Interventional Cardiac Electrophysiology, 1, 41–48.
Tavernier, R., Gevaert, S., De Sutter, J., De Clercq, A., Rottiers, H., Jordaens, L., et al. (2001). Long term results of cardioverter-defibrillator in patients with right ventricular dysplasia and malignant ventricular tachyarrhythmias. Heart, 85, 53–56.
Wichter, T., Paul, M., Wollmann, C., Acil, T., Gerdes, P., Ashraf, O., et al. (2004). Implantable cardioverter/defibrillator therapy in arrhythmogenic right ventricular cardiomyopathy: single-center experience of long-term follow-up and complications in 60 patients. Circulation, 109, 1503–1508.
Watanabe, H., Chinushi, M., Izumi, D., Sato, A., Okada, S., & Okamura, K. (2006). Decrease in amplitude of intracardiac ventricular electrogram and inappropriate therapy in patients with an implantable cardioverter defibrillator. International Heart Journal, 47, 363–370.
McKenna, W. J., Thiene, G., Nava, A., Fontaliran, F., Blomstrom-Lundqvist, C., Fontaine, G., et al. (1994). Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. British Heart Journal, 71, 215–218.
Rosenqvist, M., Beyer, T., Block, M., den Dulk, K., Minten, J., & Lindemans, F. (1998). Circulation, 98, 663–670.
Grimm, W., Flores, B. F., & Marchlinski, F. E. (1993). Complications of implantable cardioverter therapy: follow-up of 241 patients. Pacing and Clinical Electrophysiology, 16, 218–222.
Molina, J. E. (1996). Perforation of the right ventricle by transvenous defibrillator leads: prevention and treatment. Pacing and Clinical Electrophysiology, 19, 288–292.
Conflict of interest
None declared
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mugnai, G., Tomei, R., Dugo, C. et al. Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular cardiomyopathy: the course of electronic parameters, clinical features, and complications during long-term follow-up. J Interv Card Electrophysiol 41, 23–29 (2014). https://doi.org/10.1007/s10840-014-9920-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10840-014-9920-0