Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy

Barry J. Maron, Paolo Spirito, Michael J. Ackerman, Susan A. Casey, Christopher Semsarian, N. A Mark Estes, Kevin M. Shannon, Euan A. Ashley, Sharlene M. Day, Giuseppe Pacileo, Francesco Formisano, Emmanuela Devoto, Aristidis Anastasakis, J. Martijn Bos, Anna Woo, Camillo Autore, Robert H. Pass, Giuseppe Boriani, Ross F. Garberich, Adrian K. Almquist & 5 others Mark W. Russell, Luca Boni, Stuart Berger, Martin S. Maron, Mark S. Link

Research output: Contribution to journalArticle

128 Citations (Scopus)

Abstract

Objectives: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. Conclusions: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.

Original languageEnglish (US)
Pages (from-to)1527-1535
Number of pages9
JournalJournal of the American College of Cardiology
Volume61
Issue number14
DOIs
StatePublished - Apr 9 2013

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Implantable Defibrillators
Hypertrophic Cardiomyopathy
Sudden Cardiac Death
Primary Prevention
Sudden Death
Left Ventricular Hypertrophy
Ventricular Fibrillation
Ventricular Tachycardia
Defibrillators
Secondary Prevention
Heart Arrest
Tachycardia
Canada
Registries
Cause of Death
Shock
Referral and Consultation
Age Groups
Pediatrics
Equipment and Supplies

Keywords

  • cardiomyopathy
  • children
  • defibrillators
  • sudden death
  • ventricular fibrillation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy. / Maron, Barry J.; Spirito, Paolo; Ackerman, Michael J.; Casey, Susan A.; Semsarian, Christopher; Estes, N. A Mark; Shannon, Kevin M.; Ashley, Euan A.; Day, Sharlene M.; Pacileo, Giuseppe; Formisano, Francesco; Devoto, Emmanuela; Anastasakis, Aristidis; Bos, J. Martijn; Woo, Anna; Autore, Camillo; Pass, Robert H.; Boriani, Giuseppe; Garberich, Ross F.; Almquist, Adrian K.; Russell, Mark W.; Boni, Luca; Berger, Stuart; Maron, Martin S.; Link, Mark S.

In: Journal of the American College of Cardiology, Vol. 61, No. 14, 09.04.2013, p. 1527-1535.

Research output: Contribution to journalArticle

Maron, BJ, Spirito, P, Ackerman, MJ, Casey, SA, Semsarian, C, Estes, NAM, Shannon, KM, Ashley, EA, Day, SM, Pacileo, G, Formisano, F, Devoto, E, Anastasakis, A, Bos, JM, Woo, A, Autore, C, Pass, RH, Boriani, G, Garberich, RF, Almquist, AK, Russell, MW, Boni, L, Berger, S, Maron, MS & Link, MS 2013, 'Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy', Journal of the American College of Cardiology, vol. 61, no. 14, pp. 1527-1535. https://doi.org/10.1016/j.jacc.2013.01.037
Maron, Barry J. ; Spirito, Paolo ; Ackerman, Michael J. ; Casey, Susan A. ; Semsarian, Christopher ; Estes, N. A Mark ; Shannon, Kevin M. ; Ashley, Euan A. ; Day, Sharlene M. ; Pacileo, Giuseppe ; Formisano, Francesco ; Devoto, Emmanuela ; Anastasakis, Aristidis ; Bos, J. Martijn ; Woo, Anna ; Autore, Camillo ; Pass, Robert H. ; Boriani, Giuseppe ; Garberich, Ross F. ; Almquist, Adrian K. ; Russell, Mark W. ; Boni, Luca ; Berger, Stuart ; Maron, Martin S. ; Link, Mark S. / Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy. In: Journal of the American College of Cardiology. 2013 ; Vol. 61, No. 14. pp. 1527-1535.
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abstract = "Objectives: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19{\%}) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5{\%} per year overall, 14.0{\%} per year for secondary prevention after cardiac arrest, and 3.1{\%} per year for primary prevention on the basis of risk factors (5-year cumulative probability 17{\%}). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14{\%}], 10 of 71 [14{\%}], and 4 of 29 [14{\%}], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65{\%}]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41{\%}) at 17 ± 5 years of age. Conclusions: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.",
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T1 - Prevention of sudden cardiac death with implantable cardioverter- defibrillators in children and adolescents with hypertrophic cardiomyopathy

AU - Maron, Barry J.

AU - Spirito, Paolo

AU - Ackerman, Michael J.

AU - Casey, Susan A.

AU - Semsarian, Christopher

AU - Estes, N. A Mark

AU - Shannon, Kevin M.

AU - Ashley, Euan A.

AU - Day, Sharlene M.

AU - Pacileo, Giuseppe

AU - Formisano, Francesco

AU - Devoto, Emmanuela

AU - Anastasakis, Aristidis

AU - Bos, J. Martijn

AU - Woo, Anna

AU - Autore, Camillo

AU - Pass, Robert H.

AU - Boriani, Giuseppe

AU - Garberich, Ross F.

AU - Almquist, Adrian K.

AU - Russell, Mark W.

AU - Boni, Luca

AU - Berger, Stuart

AU - Maron, Martin S.

AU - Link, Mark S.

PY - 2013/4/9

Y1 - 2013/4/9

N2 - Objectives: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. Conclusions: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.

AB - Objectives: The aim of this study was to determine the efficacy of implantable cardioverter-defibrillators (ICDs) in children and adolescents with hypertrophic cardiomyopathy (HCM). Background: HCM is the most common cause of sudden death in the young. The availability of ICDs over the past decade for HCM has demonstrated the potential for sudden death prevention, predominantly in adult patients. Methods: A multicenter international registry of ICDs implanted (1987 to 2011) in 224 unrelated children and adolescents with HCM judged at high risk for sudden death was assembled. Patients received ICDs for primary (n = 188) or secondary (n = 36) prevention after undergoing evaluation at 22 referral and nonreferral institutions in the United States, Canada, Europe, and Australia. Results: Defibrillators were activated appropriately to terminate ventricular tachycardia or ventricular fibrillation in 43 of 224 patients (19%) over a mean of 4.3 ± 3.3 years. ICD intervention rates were 4.5% per year overall, 14.0% per year for secondary prevention after cardiac arrest, and 3.1% per year for primary prevention on the basis of risk factors (5-year cumulative probability 17%). The mean time from implantation to first appropriate discharge was 2.9 ± 2.7 years (range to 8.6 years). The primary prevention discharge rate terminating ventricular tachycardia or ventricular fibrillation was the same in patients who underwent implantation for 1, 2, or ≥3 risk factors (12 of 88 [14%], 10 of 71 [14%], and 4 of 29 [14%], respectively, p = 1.00). Extreme left ventricular hypertrophy was the most common risk factor present (alone or in combination with other markers) in patients experiencing primary prevention interventions (17 of 26 [65%]). ICD-related complications, particularly inappropriate shocks and lead malfunction, occurred in 91 patients (41%) at 17 ± 5 years of age. Conclusions: In a high-risk pediatric HCM cohort, ICD interventions terminating life-threatening ventricular tachyarrhythmias were frequent. Extreme left ventricular hypertrophy was most frequently associated with appropriate interventions. The rate of device complications adds a measure of complexity to ICD decisions in this age group.

KW - cardiomyopathy

KW - children

KW - defibrillators

KW - sudden death

KW - ventricular fibrillation

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