Open AccessImplantable Cardioverter‐Defibrillator Therapy in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: Predictors of Appropriate Therapy, Outcomes, and Complications
Author(s) -
Orgeron Gabriela M.,
James Cynthia A.,
Te Riele Anneline,
Tichnell Crystal,
Murray Brittney,
Bhonsale Aditya,
Kamel Ihab R.,
Zimmerman Stephan L.,
Judge Daniel P.,
Crosson Jane,
Tandri Harikrishna,
Calkins Hugh
Publication year - 2017
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.117.006242
Subject(s) - medicine , cardiology , arrhythmogenic right ventricular dysplasia , implantable cardioverter defibrillator , ventricular tachycardia , ventricular fibrillation , hazard ratio , cardiomyopathy , sudden cardiac death , atrial flutter , cardiac resynchronization therapy , atrial fibrillation , confidence interval , ejection fraction , heart failure
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.