Author + information
- Received July 28, 2013
- Revision received April 21, 2014
- Accepted April 21, 2014
- Published online July 15, 2014.
- Mark S. Link, MD∗∗ (, )
- Douglas Laidlaw, MD∗,
- Bronislava Polonsky, MS†,
- Wojciech Zareba, MD†,
- Scott McNitt, MS†,
- Kathleen Gear, RN‡,
- Frank Marcus, MD‡ and
- N. A. Mark Estes III, MD∗
- ∗Tufts Medical Center, Cardiac Arrhythmia Center, Boston, Massachusetts
- †Heart Research Follow-up Program, Cardiology Division, University of Rochester Medical Center, Rochester, New York
- ‡Section of Cardiology, University of Arizona, Tucson, Arizona
- ↵∗Reprint requests and correspondence:
Dr. Mark S. Link, Tufts Medical Center Box #197, 800 Washington Street, Boston, Massachusetts 02459.
Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with sudden cardiac death. However, the selection of patients for implanted cardioverter-defibrillators (ICDs), as well as programming of the ICD, is unclear.
Objectives The objective of this study was to identify predictors, characteristics, and treatment of ventricular arrhythmias in patients with ARVC.
Methods The Multidisciplinary Study of Right Ventricular Cardiomyopathy established the North American ARVC Registry and enrolled patients with a diagnosis of ARVC. Patients were followed prospectively.
Results Of 137 patients enrolled, 108 received ICDs. Forty-eight patients had 502 sustained episodes of ventricular arrhythmias, including 489 that were monomorphic and 13 that were polymorphic. In the patients with ICDs, independent predictors of ventricular arrhythmias in follow-up included spontaneous sustained ventricular arrhythmias before ICD implantation and T-wave inversions inferiorly. The only independent predictor for life-threatening arrhythmias, defined as sustained ventricular tachycardia (VT) ≥240 beats/min or ventricular fibrillation, was a younger age at enrollment. Anti-tachycardia pacing (ATP), independent of the cycle length of the VT, was successful in terminating 92% of VT episodes.
Conclusions In the North American ARVC Registry, the majority of ventricular arrhythmias in follow-up are monomorphic. Risk factors for ventricular arrhythmias were spontaneous ventricular arrhythmias before enrollment and a younger age at ICD implantation. ATP is highly successful in terminating VT, and all ICDs should be programmed for ATP, even for rapid VT.
- arrhythmogenic right ventricular cardiomyopathy
- implantable cardioverter-defibrillator
- ventricular arrhythmia
- ventricular tachycardia
This research was funded by National Institutes of Healthhttp://dx.doi.org/10.13039/100000002 grant U01-HL65594. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received July 28, 2013.
- Revision received April 21, 2014.
- Accepted April 21, 2014.
- American College of Cardiology Foundation