Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Predictors of appropriate therapy, outcomes, and complications

Gabriela M. Orgeron, Cynthia Anne James, Anneline Te Riele, Crystal Tichnell, Brittney Murray, Aditya Bhonsale, Ihab R Kamel, Stefan Zimmerman, Daniel P. Judge, Jane Crosson, Harikrishna Tandri, Hugh Calkins

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Article numbere006242
JournalJournal of the American Heart Association
Volume6
Issue number6
DOIs
StatePublished - Jun 1 2017

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Arrhythmogenic Right Ventricular Dysplasia
Implantable Defibrillators
Confidence Intervals
Ventricular Flutter
Ventricular Fibrillation
Ventricular Premature Complexes
Therapeutics
Electrophysiology
Sex Ratio
Sudden Cardiac Death
Syncope
Heart Transplantation
Ventricular Tachycardia
Cardiac Arrhythmias

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy/dysplasia
  • Implantable cardioverter defibrillator
  • Sudden cardiac death
  • Tachyarrhythmias
  • Ventricular fibrillation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular dysplasia/cardiomyopathy : Predictors of appropriate therapy, outcomes, and complications. / Orgeron, Gabriela M.; James, Cynthia Anne; Riele, Anneline Te; Tichnell, Crystal; Murray, Brittney; Bhonsale, Aditya; Kamel, Ihab R; Zimmerman, Stefan; Judge, Daniel P.; Crosson, Jane; Tandri, Harikrishna; Calkins, Hugh.

In: Journal of the American Heart Association, Vol. 6, No. 6, e006242, 01.06.2017.

Research output: Contribution to journalArticle

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title = "Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Predictors of appropriate therapy, outcomes, and complications",
abstract = "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.",
keywords = "Arrhythmogenic right ventricular cardiomyopathy/dysplasia, Implantable cardioverter defibrillator, Sudden cardiac death, Tachyarrhythmias, Ventricular fibrillation",
author = "Orgeron, {Gabriela M.} and James, {Cynthia Anne} and Riele, {Anneline Te} and Crystal Tichnell and Brittney Murray and Aditya Bhonsale and Kamel, {Ihab R} and Stefan Zimmerman and Judge, {Daniel P.} and Jane Crosson and Harikrishna Tandri and Hugh Calkins",
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doi = "10.1161/JAHA.117.006242",
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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 Anne

AU - Riele, Anneline Te

AU - Tichnell, Crystal

AU - Murray, Brittney

AU - Bhonsale, Aditya

AU - Kamel, Ihab R

AU - Zimmerman, Stefan

AU - Judge, Daniel P.

AU - Crosson, Jane

AU - Tandri, Harikrishna

AU - Calkins, Hugh

PY - 2017/6/1

Y1 - 2017/6/1

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

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DO - 10.1161/JAHA.117.006242

M3 - Article

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JO - Journal of the American Heart Association

JF - Journal of the American Heart Association

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