Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy

Barry J. Maron, Paolo Spirito, Win Kuang Shen, Tammy S. Haas, Francesco Formisano, Mark S. Link, Andrew E. Epstein, Adrian K. Almquist, James P. Daubert, Thorsten Lawrenz, Giuseppe Boriani, N. A Mark Estes, Stefano Favale, Marco Piccininno, Stephen L. Winters, Massimo Santini, Sandro Betocchi, Fernando Arribas, Mark V. Sherrid, Gianfranco Buja & 2 others Christopher Semsarian, Paolo Bruzzi

Research output: Contribution to journalArticle

541 Citations (Scopus)

Abstract

Context: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. Objective: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. Design, Setting, and Patients: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. Main Outcome Measure: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. Results: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P=.77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%). Conclusions: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

Original languageEnglish (US)
Pages (from-to)405-412
Number of pages8
JournalJournal of the American Medical Association
Volume298
Issue number4
StatePublished - Jul 25 2007

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Implantable Defibrillators
Hypertrophic Cardiomyopathy
Sudden Cardiac Death
Sudden Death
Primary Prevention
Ventricular Tachycardia
Ventricular Fibrillation
Ambulatory Electrocardiography
Defibrillators
Syncope
Left Ventricular Hypertrophy
Secondary Prevention
Heart Arrest
Tachycardia
Patient Selection
Multicenter Studies
Registries
Cardiac Arrhythmias
Shock
Referral and Consultation

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Maron, B. J., Spirito, P., Shen, W. K., Haas, T. S., Formisano, F., Link, M. S., ... Bruzzi, P. (2007). Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. Journal of the American Medical Association, 298(4), 405-412.

Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. / Maron, Barry J.; Spirito, Paolo; Shen, Win Kuang; Haas, Tammy S.; Formisano, Francesco; Link, Mark S.; Epstein, Andrew E.; Almquist, Adrian K.; Daubert, James P.; Lawrenz, Thorsten; Boriani, Giuseppe; Estes, N. A Mark; Favale, Stefano; Piccininno, Marco; Winters, Stephen L.; Santini, Massimo; Betocchi, Sandro; Arribas, Fernando; Sherrid, Mark V.; Buja, Gianfranco; Semsarian, Christopher; Bruzzi, Paolo.

In: Journal of the American Medical Association, Vol. 298, No. 4, 25.07.2007, p. 405-412.

Research output: Contribution to journalArticle

Maron, BJ, Spirito, P, Shen, WK, Haas, TS, Formisano, F, Link, MS, Epstein, AE, Almquist, AK, Daubert, JP, Lawrenz, T, Boriani, G, Estes, NAM, Favale, S, Piccininno, M, Winters, SL, Santini, M, Betocchi, S, Arribas, F, Sherrid, MV, Buja, G, Semsarian, C & Bruzzi, P 2007, 'Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy', Journal of the American Medical Association, vol. 298, no. 4, pp. 405-412.
Maron, Barry J. ; Spirito, Paolo ; Shen, Win Kuang ; Haas, Tammy S. ; Formisano, Francesco ; Link, Mark S. ; Epstein, Andrew E. ; Almquist, Adrian K. ; Daubert, James P. ; Lawrenz, Thorsten ; Boriani, Giuseppe ; Estes, N. A Mark ; Favale, Stefano ; Piccininno, Marco ; Winters, Stephen L. ; Santini, Massimo ; Betocchi, Sandro ; Arribas, Fernando ; Sherrid, Mark V. ; Buja, Gianfranco ; Semsarian, Christopher ; Bruzzi, Paolo. / Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. In: Journal of the American Medical Association. 2007 ; Vol. 298, No. 4. pp. 405-412.
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abstract = "Context: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. Objective: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. Design, Setting, and Patients: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. Main Outcome Measure: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. Results: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87{\%}]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20{\%}). Intervention rates were 10.6{\%} per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39{\%} [SD, 5{\%}]), and 3.6{\%} per year for primary prevention (5-year probability, 17{\%} [SD, 2{\%}]). Time to first appropriate discharge was up to 10 years, with a 27{\%} (SD, 7{\%}) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35{\%}) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P=.77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27{\%}). Conclusions: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.",
author = "Maron, {Barry J.} and Paolo Spirito and Shen, {Win Kuang} and Haas, {Tammy S.} and Francesco Formisano and Link, {Mark S.} and Epstein, {Andrew E.} and Almquist, {Adrian K.} and Daubert, {James P.} and Thorsten Lawrenz and Giuseppe Boriani and Estes, {N. A Mark} and Stefano Favale and Marco Piccininno and Winters, {Stephen L.} and Massimo Santini and Sandro Betocchi and Fernando Arribas and Sherrid, {Mark V.} and Gianfranco Buja and Christopher Semsarian and Paolo Bruzzi",
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T1 - Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy

AU - Maron, Barry J.

AU - Spirito, Paolo

AU - Shen, Win Kuang

AU - Haas, Tammy S.

AU - Formisano, Francesco

AU - Link, Mark S.

AU - Epstein, Andrew E.

AU - Almquist, Adrian K.

AU - Daubert, James P.

AU - Lawrenz, Thorsten

AU - Boriani, Giuseppe

AU - Estes, N. A Mark

AU - Favale, Stefano

AU - Piccininno, Marco

AU - Winters, Stephen L.

AU - Santini, Massimo

AU - Betocchi, Sandro

AU - Arribas, Fernando

AU - Sherrid, Mark V.

AU - Buja, Gianfranco

AU - Semsarian, Christopher

AU - Bruzzi, Paolo

PY - 2007/7/25

Y1 - 2007/7/25

N2 - Context: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. Objective: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. Design, Setting, and Patients: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. Main Outcome Measure: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. Results: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P=.77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%). Conclusions: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

AB - Context: Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved. Objective: To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM. Design, Setting, and Patients: Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope. Main Outcome Measure: Appropriate ICD intervention terminating ventricular tachycardia or fibrillation. Results: The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P=.77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%). Conclusions: In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.

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