Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillators in Children and Adolescents With Hypertrophic Cardiomyopathy

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:61

Article

Maron, Barry J.¹  Spirito, Paolo²  Ackerman, Michael J.³  Casey, Susan A.¹  Semsarian, Christopher^4,5  Estes, N. A. Mark, III^6,7  Shannon, Kevin M.⁸  Ashley, Euan A.^9,10  Day, Sharlene M.¹¹  Pacileo, Giuseppe¹²  Formisano, Francesco²  Devoto, Emmanuela²  Anastasakis, Aristidis¹³  Bos, J. Martijn³  Woo, Anna¹⁴  Autore, Camillo¹⁵  Pass, Robert H.¹⁶  Boriani, Giuseppe¹⁷  Garberich, Ross F.¹  Almquist, Adrian K.¹  Russell, Mark W.¹¹  Boni, Luca¹⁸  Berger, Stuart¹⁹  Maron, Martin S.^6,7  Link, Mark S.^6,7 

[1] Minneapolis Heart Inst Fdn, Hypertroph Cardiomyopathy Ctr, Minneapolis, MN 55407 USA

[2] Ente Osped Osped Galliera, Genoa, Italy

[3] Mayo Clin, Rochester, MN USA

[4] Univ Sydney, Royal Prince Alfred Hosp, Sydney, NSW 2006, Australia

[5] Univ Sydney, Centenary Inst, Sydney, NSW 2006, Australia

[6] Tufts Med Ctr, Boston, MA USA

[7] Sch Med, Boston, MA USA

[8] Univ Calif Los Angeles, Med Ctr, Los Angeles, CA 90024 USA

[9] Stanford Univ Hosp, Palo Alto, CA USA

[10] Stanford Univ, Sch Med, Palo Alto, CA 94304 USA

[11] Univ Michigan Hlth Syst, Mott Childrens Hosp, Ann Arbor, MI USA

[12] Univ Naples 2, Monaldi Hosp, Naples, Italy

[13] Univ Athens, Dept Cardiol, Athens, Greece

[14] Toronto Gen Hosp, Toronto, ON, Canada

[15] Univ Roma La Sapienza, Dept Clin & Mol Med, Rome, Italy

[16] Childrens Hosp Montefiore, Bronx, NY USA

[17] Univ Bologna, Inst Cardiol, S Orsola Malpighi Hosp, Bologna, Italy

[18] Ist Toscano Tumori AOU Careggi, Clin Trials Coordinating Ctr, Florence, Italy

[19] Childrens Hosp Wisconsin, Milwaukee, WI 53201 USA

关键词: cardiomyopathy;    children;    defibrillators;    sudden death;    ventricular fibrillation;   

DOI  :  10.1016/j.jacc.2013.01.037

来源: Elsevier

【 摘 要 】

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. (J Am Coll Cardiol 2013;61:1527-35) (C) 2013 by the American College of Cardiology Foundation

【 授权许可】

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