Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Domenico Corrado, Loira Leoni, Mark S. Link, Paolo Della Bella, Fiorenzo Gaita, Antonio Curnis, Jorge Uriarte Salerno, Diran Igidbashian, Antonio Raviele, Marcello Disertori, Gabriele Zanotto, Roberto Verlato, Giuseppe Vergara, Pietro Delise, Pietro Turrini, Cristina Basso, Franco Naccarella, Francesco Maddalena, N. A.Mark Estes, Gianfranco BujaGaetano Thiene

Research output: Contribution to journalArticle

420 Citations (Scopus)

Abstract

Background-Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). Methods and Results-We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40±15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39±25 months, 64 patients (48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (P<0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. Conclusions-In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricular stimulation outcome.

Original languageEnglish (US)
Pages (from-to)3084-3091
Number of pages8
JournalCirculation
Volume108
Issue number25
DOIs
StatePublished - Dec 23 2003

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Arrhythmogenic Right Ventricular Dysplasia
Implantable Defibrillators
Sudden Death
Ventricular Flutter
Ventricular Fibrillation
Ventricular Tachycardia
Therapeutics
Heart Arrest
Survival Rate
Hemodynamics
Anti-Arrhythmia Agents
Syncope
Tachycardia
Multicenter Studies
Cardiac Arrhythmias

Keywords

  • Cardiomyopathy
  • Death, sudden
  • Defibrillation
  • Prevention
  • Tachyarrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. / Corrado, Domenico; Leoni, Loira; Link, Mark S.; Della Bella, Paolo; Gaita, Fiorenzo; Curnis, Antonio; Salerno, Jorge Uriarte; Igidbashian, Diran; Raviele, Antonio; Disertori, Marcello; Zanotto, Gabriele; Verlato, Roberto; Vergara, Giuseppe; Delise, Pietro; Turrini, Pietro; Basso, Cristina; Naccarella, Franco; Maddalena, Francesco; Estes, N. A.Mark; Buja, Gianfranco; Thiene, Gaetano.

In: Circulation, Vol. 108, No. 25, 23.12.2003, p. 3084-3091.

Research output: Contribution to journalArticle

Corrado, D, Leoni, L, Link, MS, Della Bella, P, Gaita, F, Curnis, A, Salerno, JU, Igidbashian, D, Raviele, A, Disertori, M, Zanotto, G, Verlato, R, Vergara, G, Delise, P, Turrini, P, Basso, C, Naccarella, F, Maddalena, F, Estes, NAM, Buja, G & Thiene, G 2003, 'Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia', Circulation, vol. 108, no. 25, pp. 3084-3091. https://doi.org/10.1161/01.CIR.0000103130.33451.D2
Corrado, Domenico ; Leoni, Loira ; Link, Mark S. ; Della Bella, Paolo ; Gaita, Fiorenzo ; Curnis, Antonio ; Salerno, Jorge Uriarte ; Igidbashian, Diran ; Raviele, Antonio ; Disertori, Marcello ; Zanotto, Gabriele ; Verlato, Roberto ; Vergara, Giuseppe ; Delise, Pietro ; Turrini, Pietro ; Basso, Cristina ; Naccarella, Franco ; Maddalena, Francesco ; Estes, N. A.Mark ; Buja, Gianfranco ; Thiene, Gaetano. / Implantable Cardioverter-Defibrillator Therapy for Prevention of Sudden Death in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. In: Circulation. 2003 ; Vol. 108, No. 25. pp. 3084-3091.
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abstract = "Background-Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). Methods and Results-We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40±15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10{\%}), sustained ventricular tachycardia in 82 (62{\%}), syncope in 21 (16{\%}), and other in 16 (12{\%}). During a mean follow-up of 39±25 months, 64 patients (48{\%}) had appropriate ICD interventions, 21 (16{\%}) had inappropriate interventions, and 19 (14{\%}) had ICD-related complications. Fifty-three (83{\%}) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49{\%}, negative predictive value 54{\%}). Four patients (3{\%}) died, and 32 (24{\%}) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96{\%} compared with the ventricular fibrillation/flutter-free survival rate of 72{\%} (P<0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. Conclusions-In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricular stimulation outcome.",
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AU - Corrado, Domenico

AU - Leoni, Loira

AU - Link, Mark S.

AU - Della Bella, Paolo

AU - Gaita, Fiorenzo

AU - Curnis, Antonio

AU - Salerno, Jorge Uriarte

AU - Igidbashian, Diran

AU - Raviele, Antonio

AU - Disertori, Marcello

AU - Zanotto, Gabriele

AU - Verlato, Roberto

AU - Vergara, Giuseppe

AU - Delise, Pietro

AU - Turrini, Pietro

AU - Basso, Cristina

AU - Naccarella, Franco

AU - Maddalena, Francesco

AU - Estes, N. A.Mark

AU - Buja, Gianfranco

AU - Thiene, Gaetano

PY - 2003/12/23

Y1 - 2003/12/23

N2 - Background-Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). Methods and Results-We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40±15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39±25 months, 64 patients (48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (P<0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. Conclusions-In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricular stimulation outcome.

AB - Background-Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). Methods and Results-We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40±15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39±25 months, 64 patients (48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (P<0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. Conclusions-In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricular stimulation outcome.

KW - Cardiomyopathy

KW - Death, sudden

KW - Defibrillation

KW - Prevention

KW - Tachyarrhythmias

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