Related Experiment Video
Updated: Apr 27, 2026
Dual-Dye Optical Mapping of Hearts from RyR2R2474S Knock-In Mice of Catecholaminergic Polymorphic Ventricular Tachycardia
Published on: December 22, 2023
Ventricular arrhythmias in the North American multidisciplinary study of ARVC: predictors, characteristics, and
Mark S Link1, Douglas Laidlaw1, Bronislava Polonsky2
1Tufts Medical Center, Cardiac Arrhythmia Center, Boston, Massachusetts.
Insights
Sudden cardiac death risk in arrhythmogenic right ventricular cardiomyopathy (ARVC) is high. Predictors for ventricular arrhythmias include prior arrhythmias and younger age. Anti-tachycardia pacing effectively terminates ventricular tachycardia (VT).
Area of Science:
- Cardiology
- Electrophysiology
- Genetics
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a significant cause of sudden cardiac death.
- Optimal patient selection and device programming for implantable cardioverter-defibrillators (ICDs) in ARVC remain unclear.
Purpose of the Study:
- To identify predictors and characteristics of ventricular arrhythmias in ARVC patients.
- To evaluate treatment strategies for ventricular arrhythmias in ARVC.
Main Methods:
- Prospective follow-up of patients diagnosed with ARVC within the North American ARVC Registry.
- Analysis of ventricular arrhythmia episodes and outcomes in patients with and without ICDs.
Main Results:
- Of 137 ARVC patients, 108 received ICDs; 48 experienced 502 sustained ventricular arrhythmia episodes (489 monomorphic, 13 polymorphic).
- Independent predictors for ventricular arrhythmias were prior sustained ventricular arrhythmias and inferior T-wave inversions.
- Younger age at enrollment was the sole predictor for life-threatening arrhythmias (VT ≥240 bpm or VF).
- Anti-tachycardia pacing (ATP) successfully terminated 92% of VT episodes, irrespective of VT cycle length.
Conclusions:
- The majority of ventricular arrhythmias in ARVC follow-up are monomorphic.
- Spontaneous prior ventricular arrhythmias and younger age at ICD implantation are key risk factors.
- ATP is highly effective for VT termination, supporting its programming in all ICDs for ARVC patients, even for rapid VT.
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.
More Related Videos
10:39Rat Model of Right-Sided Cardiac Remodeling and Arrhythmia Using Pulmonary Artery Banding
Published on: August 30, 2024
10:18Generation and Characterization of Right Ventricular Myocardial Infarction Induced by Permanent Ligation of the Right Coronary Artery in Mice
Published on: February 1, 2022
Related Concept Videos
01:25ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias
01:29Disturbances in Heart Rhythm
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
01:28Mechanism of Cardiac Arrhythmias
01:18Dysrhythmias IV: Characteristics of Bradyarrhythmias
01:30Dysrhythmias V: Evaluating Dysrhythmias
01:28Dysrhythmias II: Classification of Tachyarrhythmias