Use of implantable cardioverter-defibrillator in inherited arrhythmogenic diseases: Data from Italian ICD registry for the years 2001-6

Alessandro Proclemer, Marco Ghidina, Domenico Facchin, Luca Rebellato, Domenico Corrado, Maurizio Gasparini, Dario Gregori

Research output: Contribution to journalArticle

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Abstract

Background: Previous studies have demonstrated the lifesaving role of an implantable cardioverter-defibrillator (ICD) in high-risk patients with genetic arrhythmogenic diseases. Aim: To evaluate the clinical data of patients with hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVD/C), idiopathic ventricular arrhythmia (IDIO-VA) including Brugada syndrome, short QT syndrome, and long QT syndrome (LQT) enrolled in the Italian ICD Registry in the years 2001-6. Methods: The survey collected prospectively clinical data and technical characteristics of ICD implants on the basis of EURID form. Results: The number of patients was 1,274 in the HCM group, 520 in the ARVD/C group, 460 in the IDIO-VA group, and 245 in the LQT group. Primary prevention involved 29.5% of patients in the HCM group, 15.9% in the ARVD/C group, 16.9% in the IDIO-VA group, and 16.6% in the LQT group, respectively. Ventricular tachycardia and ventricular fibrillation were reported in 49.0% and 24.8% in the HCM group, 72.9% and 20.0% in the ARVD/C group, 48.2% and 42.9% in the IDIO-VA group, and 21.2% and 61.6% in the LQT group, respectively. Single-, dual-, and triple-chamber ICDs were implanted in 41.5%, 47.6%, and 10.9% in the HCM group; 57.9%, 36.7%, and 5.4% in the ARVD/C group; 55.0%, 45.0%, and 0% in the IDIO-VA group; and 57.4%, 42.6%, and 0% in LQT group, respectively. Conclusion: ICD therapies in the years 2001-6 for patients with inherited arrhythmia syndromes were utilized in Italy in a still-limited number of patients. Secondary prevention represented the major indication for ICD implant and the majority of patients were treated by single-chamber ICDs.

Original languageEnglish
Pages (from-to)434-445
Number of pages12
JournalPACE - Pacing and Clinical Electrophysiology
Volume32
Issue number4
DOIs
Publication statusPublished - Apr 2009

Fingerprint

Arrhythmogenic Right Ventricular Dysplasia
Implantable Defibrillators
Long QT Syndrome
Registries
Hypertrophic Cardiomyopathy
Cardiac Arrhythmias
Brugada Syndrome
Inborn Genetic Diseases
Ventricular Fibrillation
Primary Prevention
Ventricular Tachycardia
Secondary Prevention
Italy

Keywords

  • Implantable cardioverter-defibrillator
  • Inherited arrhythmia syndromes
  • Registry
  • Sudden cardiac death
  • Ventricular fibrillation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Use of implantable cardioverter-defibrillator in inherited arrhythmogenic diseases : Data from Italian ICD registry for the years 2001-6. / Proclemer, Alessandro; Ghidina, Marco; Facchin, Domenico; Rebellato, Luca; Corrado, Domenico; Gasparini, Maurizio; Gregori, Dario.

In: PACE - Pacing and Clinical Electrophysiology, Vol. 32, No. 4, 04.2009, p. 434-445.

Research output: Contribution to journalArticle

Proclemer, Alessandro ; Ghidina, Marco ; Facchin, Domenico ; Rebellato, Luca ; Corrado, Domenico ; Gasparini, Maurizio ; Gregori, Dario. / Use of implantable cardioverter-defibrillator in inherited arrhythmogenic diseases : Data from Italian ICD registry for the years 2001-6. In: PACE - Pacing and Clinical Electrophysiology. 2009 ; Vol. 32, No. 4. pp. 434-445.
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abstract = "Background: Previous studies have demonstrated the lifesaving role of an implantable cardioverter-defibrillator (ICD) in high-risk patients with genetic arrhythmogenic diseases. Aim: To evaluate the clinical data of patients with hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVD/C), idiopathic ventricular arrhythmia (IDIO-VA) including Brugada syndrome, short QT syndrome, and long QT syndrome (LQT) enrolled in the Italian ICD Registry in the years 2001-6. Methods: The survey collected prospectively clinical data and technical characteristics of ICD implants on the basis of EURID form. Results: The number of patients was 1,274 in the HCM group, 520 in the ARVD/C group, 460 in the IDIO-VA group, and 245 in the LQT group. Primary prevention involved 29.5{\%} of patients in the HCM group, 15.9{\%} in the ARVD/C group, 16.9{\%} in the IDIO-VA group, and 16.6{\%} in the LQT group, respectively. Ventricular tachycardia and ventricular fibrillation were reported in 49.0{\%} and 24.8{\%} in the HCM group, 72.9{\%} and 20.0{\%} in the ARVD/C group, 48.2{\%} and 42.9{\%} in the IDIO-VA group, and 21.2{\%} and 61.6{\%} in the LQT group, respectively. Single-, dual-, and triple-chamber ICDs were implanted in 41.5{\%}, 47.6{\%}, and 10.9{\%} in the HCM group; 57.9{\%}, 36.7{\%}, and 5.4{\%} in the ARVD/C group; 55.0{\%}, 45.0{\%}, and 0{\%} in the IDIO-VA group; and 57.4{\%}, 42.6{\%}, and 0{\%} in LQT group, respectively. Conclusion: ICD therapies in the years 2001-6 for patients with inherited arrhythmia syndromes were utilized in Italy in a still-limited number of patients. Secondary prevention represented the major indication for ICD implant and the majority of patients were treated by single-chamber ICDs.",
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AU - Ghidina, Marco

AU - Facchin, Domenico

AU - Rebellato, Luca

AU - Corrado, Domenico

AU - Gasparini, Maurizio

AU - Gregori, Dario

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N2 - Background: Previous studies have demonstrated the lifesaving role of an implantable cardioverter-defibrillator (ICD) in high-risk patients with genetic arrhythmogenic diseases. Aim: To evaluate the clinical data of patients with hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVD/C), idiopathic ventricular arrhythmia (IDIO-VA) including Brugada syndrome, short QT syndrome, and long QT syndrome (LQT) enrolled in the Italian ICD Registry in the years 2001-6. Methods: The survey collected prospectively clinical data and technical characteristics of ICD implants on the basis of EURID form. Results: The number of patients was 1,274 in the HCM group, 520 in the ARVD/C group, 460 in the IDIO-VA group, and 245 in the LQT group. Primary prevention involved 29.5% of patients in the HCM group, 15.9% in the ARVD/C group, 16.9% in the IDIO-VA group, and 16.6% in the LQT group, respectively. Ventricular tachycardia and ventricular fibrillation were reported in 49.0% and 24.8% in the HCM group, 72.9% and 20.0% in the ARVD/C group, 48.2% and 42.9% in the IDIO-VA group, and 21.2% and 61.6% in the LQT group, respectively. Single-, dual-, and triple-chamber ICDs were implanted in 41.5%, 47.6%, and 10.9% in the HCM group; 57.9%, 36.7%, and 5.4% in the ARVD/C group; 55.0%, 45.0%, and 0% in the IDIO-VA group; and 57.4%, 42.6%, and 0% in LQT group, respectively. Conclusion: ICD therapies in the years 2001-6 for patients with inherited arrhythmia syndromes were utilized in Italy in a still-limited number of patients. Secondary prevention represented the major indication for ICD implant and the majority of patients were treated by single-chamber ICDs.

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KW - Sudden cardiac death

KW - Ventricular fibrillation

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