• Mark S Link, Douglas Laidlaw, Bronislava Polonsky, Wojciech Zareba, Scott McNitt, Kathleen Gear, Frank Marcus, and N A Mark Estes.
• Tufts Medical Center, Cardiac Arrhythmia Center, Boston, Massachusetts. Electronic address: mlink@tuftsmedicalcenter.org.
• J. Am. Coll. Cardiol. 2014 Jul 15; 64 (2): 119-25.

BackgroundArrhythmogenic 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.ObjectivesThe objective of this study was to identify predictors, characteristics, and treatment of ventricular arrhythmias in patients with ARVC.MethodsThe Multidisciplinary Study of Right Ventricular Cardiomyopathy established the North American ARVC Registry and enrolled patients with a diagnosis of ARVC. Patients were followed prospectively.ResultsOf 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.ConclusionsIn 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.Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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