Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy

Barry J. Maron, Win Kuang Shen, Mark S. Link, Andrew E. Epstein, Adrian K. Almquist, James P. Daubert, Gust H. Bardy, Stefano Favale, Robert F. Rea, Giuseppe Boriani, N. A.Mark Estes, Paolo Spirito, Susan A. Casey, Marshall S. Stanton, Sandro Betocchi

Research output: Contribution to journalArticle

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Abstract

Background: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. Methods: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death. Results: At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [±SD], 40±16), and 69 patients (54 percent) were less than 41 years old. The average follow- up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation. Conclusions: Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death.

Original languageEnglish (US)
Pages (from-to)365-373
Number of pages9
JournalNew England Journal of Medicine
Volume342
Issue number6
DOIs
StatePublished - Feb 10 2000

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Implantable Defibrillators
Hypertrophic Cardiomyopathy
Sudden Death
Defibrillators
Ventricular Tachycardia
Ventricular Fibrillation
Primary Prevention
Secondary Prevention
Equipment and Supplies
Inborn Genetic Diseases
Heart Arrest
Tachycardia
Multicenter Studies
Cardiac Arrhythmias
Shock
Retrospective Studies

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. / Maron, Barry J.; Shen, Win Kuang; Link, Mark S.; Epstein, Andrew E.; Almquist, Adrian K.; Daubert, James P.; Bardy, Gust H.; Favale, Stefano; Rea, Robert F.; Boriani, Giuseppe; Estes, N. A.Mark; Spirito, Paolo; Casey, Susan A.; Stanton, Marshall S.; Betocchi, Sandro.

In: New England Journal of Medicine, Vol. 342, No. 6, 10.02.2000, p. 365-373.

Research output: Contribution to journalArticle

Maron, BJ, Shen, WK, Link, MS, Epstein, AE, Almquist, AK, Daubert, JP, Bardy, GH, Favale, S, Rea, RF, Boriani, G, Estes, NAM, Spirito, P, Casey, SA, Stanton, MS & Betocchi, S 2000, 'Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy', New England Journal of Medicine, vol. 342, no. 6, pp. 365-373. https://doi.org/10.1056/NEJM200002103420601
Maron, Barry J. ; Shen, Win Kuang ; Link, Mark S. ; Epstein, Andrew E. ; Almquist, Adrian K. ; Daubert, James P. ; Bardy, Gust H. ; Favale, Stefano ; Rea, Robert F. ; Boriani, Giuseppe ; Estes, N. A.Mark ; Spirito, Paolo ; Casey, Susan A. ; Stanton, Marshall S. ; Betocchi, Sandro. / Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. In: New England Journal of Medicine. 2000 ; Vol. 342, No. 6. pp. 365-373.
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abstract = "Background: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. Methods: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death. Results: At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [±SD], 40±16), and 69 patients (54 percent) were less than 41 years old. The average follow- up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation. Conclusions: Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death.",
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T1 - Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy

AU - Maron, Barry J.

AU - Shen, Win Kuang

AU - Link, Mark S.

AU - Epstein, Andrew E.

AU - Almquist, Adrian K.

AU - Daubert, James P.

AU - Bardy, Gust H.

AU - Favale, Stefano

AU - Rea, Robert F.

AU - Boriani, Giuseppe

AU - Estes, N. A.Mark

AU - Spirito, Paolo

AU - Casey, Susan A.

AU - Stanton, Marshall S.

AU - Betocchi, Sandro

PY - 2000/2/10

Y1 - 2000/2/10

N2 - Background: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. Methods: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death. Results: At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [±SD], 40±16), and 69 patients (54 percent) were less than 41 years old. The average follow- up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation. Conclusions: Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death.

AB - Background: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. Methods: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death. Results: At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [±SD], 40±16), and 69 patients (54 percent) were less than 41 years old. The average follow- up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation. Conclusions: Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death.

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