Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Predictors of appropriate therapy,outcomes,and complications

Background-arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. once the diagnosis is established,risk stratification to determine whether implantable cardioverter-defibrillator (icd) placement is warranted is critical. methods and results-the cohort included 312 patients (163 men,age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an icd. over 8.8±7.33 years,186 participants (60%) had appropriate icd therapy and 58 (19%) had an intervention for ventricular fibrillation/flutter. ventricular tachycardia at presentation (hazard ratio [hr]: 1.86; 95% confidence interval [ci],1.38-2.49; p < 0.001),inducibility on electrophysiology study (hr: 3.14; 95% ci,1.95-5.05; p < 0.001),male sex (hr: 1.62; 95% ci,1.20-2.19; p = 0.001),inverted t waves in = 3 precordial leads (hr: 1.66; 95% ci,1.09-2.52; p = 0.018),and premature ventricular contraction count = 1000/24 hours (hr: 2.30; 95% ci,1.32- 4.00; p = 0.003) were predictors of any appropriate icd therapy. inducibility at electrophysiology study (hr: 2.28; 95% ci,1.10- 4.70; p = 0.025) remained as the only predictor after multivariable analysis. the predictors for ventricular fibrillation/flutter were premature ventricular contraction = 1000/24 hours (hr: 4.39; 95% ci,1.32-14.61; p = 0.016),syncope (hr: 1.85; 95% ci,1.10- 3.11; p = 0.021),aged = 30 years at presentation (hr: 1.76; 95% ci,1.04-3.00; p < 0.036),and male sex (hr: 1.73; 95% ci,1.01- 2.97; p = 0.046). younger age at presentation (hr: 3.14; 95% ci,1.32-7.48; p = 0.010) and high premature ventricular contraction burden (hr: 4.43; 95% ci,1.35-14.57; p < 0.014) remained as independent predictors of ventricular fibrillation/flutter. complications occurred in 66 participants (21%),and 64 (21%) had inappropriate icd interventions. overall mortality was low at 2%,and 4% underwent heart transplantation. conclusion-these findings represent an important step in identifying predictors of icd therapy for potentially fatal ventricular fibrillation/flutter and should be considered when developing a risk stratification model for arrhythmogenic right ventricular dysplasia/cardiomyopathy. © 2017 the authors.