TY - JOUR
T1 - Implantable Cardioverter-Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy
T2 - Predictors of Appropriate Therapy, Outcomes, and Complications
AU - Orgeron, Gabriela M
AU - James, Cynthia A.
AU - Te Riele, Anneline
AU - Tichnell, Crystal
AU - Murray, Brittney
AU - Bhonsale, Aditya
AU - Kamel, Ihab R.
AU - Zimmerman, Stephan L
AU - Judge, Daniel P.
AU - Crosson, Jane
AU - Tandri, Harikrishna
AU - Calkins, Hugh
N1 - Publisher Copyright:
© 2017 The Authors.
PY - 2017/6/6
Y1 - 2017/6/6
N2 - 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.
AB - 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.
KW - Arrhythmogenic right ventricular cardiomyopathy/dysplasia
KW - Implantable cardioverter defibrillator
KW - Sudden cardiac death
KW - Tachyarrhythmias
KW - Ventricular fibrillation
U2 - 10.1161/JAHA.117.006242
DO - 10.1161/JAHA.117.006242
M3 - Article
C2 - 28588093
SN - 2047-9980
VL - 6
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 6
M1 - e006242
ER -