Performance of the 2015 International Task Force Consensus Statement Risk Stratification Algorithm for Implantable Cardioverter-Defibrillator Placement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Circ Arrhythm Electrophysiol. 2018 Feb;11(2):e005593. doi: 10.1161/CIRCEP.117.005593. Epub 2018 Feb 16.


Background: Ventricular arrhythmias are a feared complication of arrhythmogenic right ventricular dysplasia/cardiomyopathy. In 2015, an International Task Force Consensus Statement proposed a risk stratification algorithm for implantable cardioverter-defibrillator placement in arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Methods and results: To evaluate performance of the algorithm, 365 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients were classified as having a Class I, IIa, IIb, or III indication per the algorithm at baseline. Survival free from sustained ventricular arrhythmia (VT/VF) in follow-up was the primary outcome. Incidence of ventricular fibrillation/flutter cycle length <240 ms was also assessed. Two hundred twenty-four (61%) patients had a Class I implantable cardioverter-defibrillator indication; 80 (22%), Class IIa; 54 (15%), Class IIb; and 7 (2%), Class III. During a median 4.2 (interquartile range, 1.7-8.4)-year follow-up, 190 (52%) patients had VT/VF and 60 (16%) had ventricular fibrillation/flutter. Although the algorithm appropriately differentiated risk of VT/VF, incidence of VT/VF was underestimated (observed versus expected: 29.6 [95% confidence interval, 25.2-34.0] versus >10%/year Class I; 15.5 [confidence interval 11.1-21.6] versus 1% to 10%/year Class IIa). In addition, the algorithm did not differentiate survival free from ventricular fibrillation/flutter between Class I and IIa patients (P=0.97) or for VT/VF in Class I and IIa primary prevention patients (P=0.22). Adding Holter results (<1000 premature ventricular contractions/24 hours) to International Task Force Consensus classification differentiated risks.

Conclusions: While the algorithm differentiates arrhythmic risk well overall, it did not distinguish ventricular fibrillation/flutter risks of patients with Class I and IIa implantable cardioverter-defibrillator indications. Limited differentiation was seen for primary prevention cases. As these are vital uncertainties in clinical decision-making, refinements to the algorithm are suggested prior to implementation.

Keywords: arrhythmia; arrhythmogenic right ventricular dysplasia/cardiomyopathy; implantable cardioverter-defibrillator; ventricular fibrillation; ventricular tachycardia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Algorithms*
  • Arrhythmogenic Right Ventricular Dysplasia / complications
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology
  • Arrhythmogenic Right Ventricular Dysplasia / therapy*
  • Consensus*
  • Defibrillators, Implantable*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Primary Prevention / standards*
  • Prospective Studies
  • Registries
  • Risk Assessment / methods*
  • Risk Factors
  • United States / epidemiology
  • Ventricular Fibrillation / epidemiology
  • Ventricular Fibrillation / etiology
  • Ventricular Fibrillation / prevention & control*