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

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...

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Veröffentlicht in:Journal of the American College of Cardiology 2013-04, Vol.61 (14), p.1527-1535
Hauptverfasser: Maron, Barry J., MD, Spirito, Paolo, MD, Ackerman, Michael J., MD, PhD, Casey, Susan A., RN, Semsarian, Christopher, MB, BS, PhD, Estes, N.A. Mark, MD, Shannon, Kevin M., MD, Ashley, Euan A., PhD, Day, Sharlene M., MD, Pacileo, Giuseppe, MD, Formisano, Francesco, MD, Devoto, Emmanuela, MD, Anastasakis, Aristidis, MD, Bos, J. Martijn, MD, PhD, Woo, Anna, MD, Autore, Camillo, MD, Pass, Robert H., MD, Boriani, Giuseppe, MD, PhD, Garberich, Ross F., MS, Almquist, Adrian K., MD, Russell, Mark W., MD, Boni, Luca, MD, PhD, Berger, Stuart, MD, Maron, Martin S., MD, Link, Mark S., MD
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container_end_page 1535
container_issue 14
container_start_page 1527
container_title Journal of the American College of Cardiology
container_volume 61
creator Maron, Barry J., MD
Spirito, Paolo, MD
Ackerman, Michael J., MD, PhD
Casey, Susan A., RN
Semsarian, Christopher, MB, BS, PhD
Estes, N.A. Mark, MD
Shannon, Kevin M., MD
Ashley, Euan A., PhD
Day, Sharlene M., MD
Pacileo, Giuseppe, MD
Formisano, Francesco, MD
Devoto, Emmanuela, MD
Anastasakis, Aristidis, MD
Bos, J. Martijn, MD, PhD
Woo, Anna, MD
Autore, Camillo, MD
Pass, Robert H., MD
Boriani, Giuseppe, MD, PhD
Garberich, Ross F., MS
Almquist, Adrian K., MD
Russell, Mark W., MD
Boni, Luca, MD, PhD
Berger, Stuart, MD
Maron, Martin S., MD
Link, Mark S., MD
description 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.
doi_str_mv 10.1016/j.jacc.2013.01.037
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Mark, MD ; Shannon, Kevin M., MD ; Ashley, Euan A., PhD ; Day, Sharlene M., MD ; Pacileo, Giuseppe, MD ; Formisano, Francesco, MD ; Devoto, Emmanuela, MD ; Anastasakis, Aristidis, MD ; Bos, J. Martijn, MD, PhD ; Woo, Anna, MD ; Autore, Camillo, MD ; Pass, Robert H., MD ; Boriani, Giuseppe, MD, PhD ; Garberich, Ross F., MS ; Almquist, Adrian K., MD ; Russell, Mark W., MD ; Boni, Luca, MD, PhD ; Berger, Stuart, MD ; Maron, Martin S., MD ; Link, Mark S., MD</creator><creatorcontrib>Maron, Barry J., MD ; Spirito, Paolo, MD ; Ackerman, Michael J., MD, PhD ; Casey, Susan A., RN ; Semsarian, Christopher, MB, BS, PhD ; Estes, N.A. Mark, MD ; Shannon, Kevin M., MD ; Ashley, Euan A., PhD ; Day, Sharlene M., MD ; Pacileo, Giuseppe, MD ; Formisano, Francesco, MD ; Devoto, Emmanuela, MD ; Anastasakis, Aristidis, MD ; Bos, J. Martijn, MD, PhD ; Woo, Anna, MD ; Autore, Camillo, MD ; Pass, Robert H., MD ; Boriani, Giuseppe, MD, PhD ; Garberich, Ross F., MS ; Almquist, Adrian K., MD ; Russell, Mark W., MD ; Boni, Luca, MD, PhD ; Berger, Stuart, MD ; Maron, Martin S., MD ; Link, Mark S., MD</creatorcontrib><description>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.</description><identifier>ISSN: 0735-1097</identifier><identifier>EISSN: 1558-3597</identifier><identifier>DOI: 10.1016/j.jacc.2013.01.037</identifier><identifier>PMID: 23500286</identifier><identifier>CODEN: JACCDI</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adolescent ; Age ; Age Factors ; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy ; Australia ; Biological and medical sciences ; Blood pressure ; Canada ; Cardiology ; Cardiology. Vascular system ; Cardiomyopathy ; Cardiomyopathy, Hypertrophic - complications ; Cardiomyopathy, Hypertrophic - diagnosis ; Cardiomyopathy, Hypertrophic - mortality ; Cardiomyopathy, Hypertrophic - therapy ; Cardiovascular ; Cause of Death ; Chi-Square Distribution ; Child ; Child, Preschool ; children ; Confidence Intervals ; Death, Sudden, Cardiac - etiology ; Death, Sudden, Cardiac - prevention &amp; control ; Defibrillators ; Defibrillators, Implantable - utilization ; Disease prevention ; Electrocardiography - methods ; Emergency and intensive care: neonates and children. Prematurity. Sudden death ; Europe ; Family medical history ; Female ; Follow-Up Studies ; Heart ; Humans ; Intensive care medicine ; Internal Medicine ; Intervention ; Kaplan-Meier Estimate ; Male ; Medical sciences ; Myocarditis. Cardiomyopathies ; Pediatrics ; Proportional Hazards Models ; Registries ; Risk Assessment ; Risk factors ; Severity of Illness Index ; Sex Factors ; sudden death ; Survival Analysis ; Teenagers ; Time Factors ; Treatment Outcome ; United States ; ventricular fibrillation</subject><ispartof>Journal of the American College of Cardiology, 2013-04, Vol.61 (14), p.1527-1535</ispartof><rights>American College of Cardiology Foundation</rights><rights>2013 American College of Cardiology Foundation</rights><rights>2014 INIST-CNRS</rights><rights>Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.</rights><rights>Copyright Elsevier Limited Apr 9, 2013</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c513t-8aa7bf783615554e9d980f62d63fcc17aa5452d5e27b0a93add3ae4b9eced6313</citedby><cites>FETCH-LOGICAL-c513t-8aa7bf783615554e9d980f62d63fcc17aa5452d5e27b0a93add3ae4b9eced6313</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0735109713004762$$EHTML$$P50$$Gelsevier$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=27238986$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23500286$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Maron, Barry J., MD</creatorcontrib><creatorcontrib>Spirito, Paolo, MD</creatorcontrib><creatorcontrib>Ackerman, Michael J., MD, PhD</creatorcontrib><creatorcontrib>Casey, Susan A., RN</creatorcontrib><creatorcontrib>Semsarian, Christopher, MB, BS, PhD</creatorcontrib><creatorcontrib>Estes, N.A. Mark, MD</creatorcontrib><creatorcontrib>Shannon, Kevin M., MD</creatorcontrib><creatorcontrib>Ashley, Euan A., PhD</creatorcontrib><creatorcontrib>Day, Sharlene M., MD</creatorcontrib><creatorcontrib>Pacileo, Giuseppe, MD</creatorcontrib><creatorcontrib>Formisano, Francesco, MD</creatorcontrib><creatorcontrib>Devoto, Emmanuela, MD</creatorcontrib><creatorcontrib>Anastasakis, Aristidis, MD</creatorcontrib><creatorcontrib>Bos, J. Martijn, MD, PhD</creatorcontrib><creatorcontrib>Woo, Anna, MD</creatorcontrib><creatorcontrib>Autore, Camillo, MD</creatorcontrib><creatorcontrib>Pass, Robert H., MD</creatorcontrib><creatorcontrib>Boriani, Giuseppe, MD, PhD</creatorcontrib><creatorcontrib>Garberich, Ross F., MS</creatorcontrib><creatorcontrib>Almquist, Adrian K., MD</creatorcontrib><creatorcontrib>Russell, Mark W., MD</creatorcontrib><creatorcontrib>Boni, Luca, MD, PhD</creatorcontrib><creatorcontrib>Berger, Stuart, MD</creatorcontrib><creatorcontrib>Maron, Martin S., MD</creatorcontrib><creatorcontrib>Link, Mark S., MD</creatorcontrib><title>Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillators in Children and Adolescents With Hypertrophic Cardiomyopathy</title><title>Journal of the American College of Cardiology</title><addtitle>J Am Coll Cardiol</addtitle><description>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.</description><subject>Adolescent</subject><subject>Age</subject><subject>Age Factors</subject><subject>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</subject><subject>Australia</subject><subject>Biological and medical sciences</subject><subject>Blood pressure</subject><subject>Canada</subject><subject>Cardiology</subject><subject>Cardiology. Vascular system</subject><subject>Cardiomyopathy</subject><subject>Cardiomyopathy, Hypertrophic - complications</subject><subject>Cardiomyopathy, Hypertrophic - diagnosis</subject><subject>Cardiomyopathy, Hypertrophic - mortality</subject><subject>Cardiomyopathy, Hypertrophic - therapy</subject><subject>Cardiovascular</subject><subject>Cause of Death</subject><subject>Chi-Square Distribution</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>children</subject><subject>Confidence Intervals</subject><subject>Death, Sudden, Cardiac - etiology</subject><subject>Death, Sudden, Cardiac - prevention &amp; control</subject><subject>Defibrillators</subject><subject>Defibrillators, Implantable - utilization</subject><subject>Disease prevention</subject><subject>Electrocardiography - methods</subject><subject>Emergency and intensive care: neonates and children. Prematurity. Sudden death</subject><subject>Europe</subject><subject>Family medical history</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Heart</subject><subject>Humans</subject><subject>Intensive care medicine</subject><subject>Internal Medicine</subject><subject>Intervention</subject><subject>Kaplan-Meier Estimate</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Myocarditis. Cardiomyopathies</subject><subject>Pediatrics</subject><subject>Proportional Hazards Models</subject><subject>Registries</subject><subject>Risk Assessment</subject><subject>Risk factors</subject><subject>Severity of Illness Index</subject><subject>Sex Factors</subject><subject>sudden death</subject><subject>Survival Analysis</subject><subject>Teenagers</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><subject>United States</subject><subject>ventricular fibrillation</subject><issn>0735-1097</issn><issn>1558-3597</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9ks2K1TAYhoMoznH0BlxIQQQ3rflpmhZEGM6oMzCgMIrLkCZfOaltU5P2QO_BizadHh2YhZtkked9v583CL0kOCOYFO_arFVaZxQTlmGSYSYeoR3hvEwZr8RjtMOC8ZTgSpyhZyG0GOOiJNVTdEYZx5iWxQ79_urhCMNk3ZC4JrmdjYEh2StvrNLJJajpkPyw8bjux04Nk6o72J7dEfwEPr2Extbedp2anA-JjeqD7YyPNmowyYVxHQQdS4TN6GoZo9C78WD1yalf3BgLLc_Rk0Z1AV6c7nP0_dPHb_ur9ObL5-v9xU2qOWFTWiol6kaUrIjD8hwqU5W4KagpWKM1EUrxnFPDgYoaq4opY5iCvK5AQ2QIO0dvN9_Ru18zhEn2NrYYRxjAzUESRnNWEUpZRF8_QFs3-yF2J0kRq8Qt3lF0o7R3IXho5Ohtr_wiCZZrVrKVa1ZyzUpiImNWUfTqZD3XPZh_kr_hRODNCVBBq67xatA23HOCsrK6495vHMSdHS14GbSFIQ5rPehJGmf_38eHB3Ld2cHGij9hgXA_rwxUYnm7_qr1UxGGcS4Kyv4A5J7Izg</recordid><startdate>20130409</startdate><enddate>20130409</enddate><creator>Maron, Barry J., MD</creator><creator>Spirito, Paolo, MD</creator><creator>Ackerman, Michael J., MD, PhD</creator><creator>Casey, Susan A., RN</creator><creator>Semsarian, Christopher, MB, BS, PhD</creator><creator>Estes, N.A. Mark, MD</creator><creator>Shannon, Kevin M., MD</creator><creator>Ashley, Euan A., PhD</creator><creator>Day, Sharlene M., MD</creator><creator>Pacileo, Giuseppe, MD</creator><creator>Formisano, Francesco, MD</creator><creator>Devoto, Emmanuela, MD</creator><creator>Anastasakis, Aristidis, MD</creator><creator>Bos, J. Martijn, MD, PhD</creator><creator>Woo, Anna, MD</creator><creator>Autore, Camillo, MD</creator><creator>Pass, Robert H., MD</creator><creator>Boriani, Giuseppe, MD, PhD</creator><creator>Garberich, Ross F., MS</creator><creator>Almquist, Adrian K., MD</creator><creator>Russell, Mark W., MD</creator><creator>Boni, Luca, MD, PhD</creator><creator>Berger, Stuart, MD</creator><creator>Maron, Martin S., MD</creator><creator>Link, Mark S., MD</creator><general>Elsevier Inc</general><general>Elsevier</general><general>Elsevier Limited</general><scope>6I.</scope><scope>AAFTH</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>20130409</creationdate><title>Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillators in Children and Adolescents With Hypertrophic Cardiomyopathy</title><author>Maron, Barry J., MD ; Spirito, Paolo, MD ; Ackerman, Michael J., MD, PhD ; Casey, Susan A., RN ; Semsarian, Christopher, MB, BS, PhD ; Estes, N.A. Mark, MD ; Shannon, Kevin M., MD ; Ashley, Euan A., PhD ; Day, Sharlene M., MD ; Pacileo, Giuseppe, MD ; Formisano, Francesco, MD ; Devoto, Emmanuela, MD ; Anastasakis, Aristidis, MD ; Bos, J. Martijn, MD, PhD ; Woo, Anna, MD ; Autore, Camillo, MD ; Pass, Robert H., MD ; Boriani, Giuseppe, MD, PhD ; Garberich, Ross F., MS ; Almquist, Adrian K., MD ; Russell, Mark W., MD ; Boni, Luca, MD, PhD ; Berger, Stuart, MD ; Maron, Martin S., MD ; Link, Mark S., MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c513t-8aa7bf783615554e9d980f62d63fcc17aa5452d5e27b0a93add3ae4b9eced6313</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adolescent</topic><topic>Age</topic><topic>Age Factors</topic><topic>Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy</topic><topic>Australia</topic><topic>Biological and medical sciences</topic><topic>Blood pressure</topic><topic>Canada</topic><topic>Cardiology</topic><topic>Cardiology. Vascular system</topic><topic>Cardiomyopathy</topic><topic>Cardiomyopathy, Hypertrophic - complications</topic><topic>Cardiomyopathy, Hypertrophic - diagnosis</topic><topic>Cardiomyopathy, Hypertrophic - mortality</topic><topic>Cardiomyopathy, Hypertrophic - therapy</topic><topic>Cardiovascular</topic><topic>Cause of Death</topic><topic>Chi-Square Distribution</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>children</topic><topic>Confidence Intervals</topic><topic>Death, Sudden, Cardiac - etiology</topic><topic>Death, Sudden, Cardiac - prevention &amp; control</topic><topic>Defibrillators</topic><topic>Defibrillators, Implantable - utilization</topic><topic>Disease prevention</topic><topic>Electrocardiography - methods</topic><topic>Emergency and intensive care: neonates and children. Prematurity. Sudden death</topic><topic>Europe</topic><topic>Family medical history</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Heart</topic><topic>Humans</topic><topic>Intensive care medicine</topic><topic>Internal Medicine</topic><topic>Intervention</topic><topic>Kaplan-Meier Estimate</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Myocarditis. Cardiomyopathies</topic><topic>Pediatrics</topic><topic>Proportional Hazards Models</topic><topic>Registries</topic><topic>Risk Assessment</topic><topic>Risk factors</topic><topic>Severity of Illness Index</topic><topic>Sex Factors</topic><topic>sudden death</topic><topic>Survival Analysis</topic><topic>Teenagers</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><topic>United States</topic><topic>ventricular fibrillation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Maron, Barry J., MD</creatorcontrib><creatorcontrib>Spirito, Paolo, MD</creatorcontrib><creatorcontrib>Ackerman, Michael J., MD, PhD</creatorcontrib><creatorcontrib>Casey, Susan A., RN</creatorcontrib><creatorcontrib>Semsarian, Christopher, MB, BS, PhD</creatorcontrib><creatorcontrib>Estes, N.A. 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Martijn, MD, PhD</creatorcontrib><creatorcontrib>Woo, Anna, MD</creatorcontrib><creatorcontrib>Autore, Camillo, MD</creatorcontrib><creatorcontrib>Pass, Robert H., MD</creatorcontrib><creatorcontrib>Boriani, Giuseppe, MD, PhD</creatorcontrib><creatorcontrib>Garberich, Ross F., MS</creatorcontrib><creatorcontrib>Almquist, Adrian K., MD</creatorcontrib><creatorcontrib>Russell, Mark W., MD</creatorcontrib><creatorcontrib>Boni, Luca, MD, PhD</creatorcontrib><creatorcontrib>Berger, Stuart, MD</creatorcontrib><creatorcontrib>Maron, Martin S., MD</creatorcontrib><creatorcontrib>Link, Mark S., MD</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>Nursing &amp; Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the American College of Cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Maron, Barry J., MD</au><au>Spirito, Paolo, MD</au><au>Ackerman, Michael J., MD, PhD</au><au>Casey, Susan A., RN</au><au>Semsarian, Christopher, MB, BS, PhD</au><au>Estes, N.A. Mark, MD</au><au>Shannon, Kevin M., MD</au><au>Ashley, Euan A., PhD</au><au>Day, Sharlene M., MD</au><au>Pacileo, Giuseppe, MD</au><au>Formisano, Francesco, MD</au><au>Devoto, Emmanuela, MD</au><au>Anastasakis, Aristidis, MD</au><au>Bos, J. Martijn, MD, PhD</au><au>Woo, Anna, MD</au><au>Autore, Camillo, MD</au><au>Pass, Robert H., MD</au><au>Boriani, Giuseppe, MD, PhD</au><au>Garberich, Ross F., MS</au><au>Almquist, Adrian K., MD</au><au>Russell, Mark W., MD</au><au>Boni, Luca, MD, PhD</au><au>Berger, Stuart, MD</au><au>Maron, Martin S., MD</au><au>Link, Mark S., MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillators in Children and Adolescents With Hypertrophic Cardiomyopathy</atitle><jtitle>Journal of the American College of Cardiology</jtitle><addtitle>J Am Coll Cardiol</addtitle><date>2013-04-09</date><risdate>2013</risdate><volume>61</volume><issue>14</issue><spage>1527</spage><epage>1535</epage><pages>1527-1535</pages><issn>0735-1097</issn><eissn>1558-3597</eissn><coden>JACCDI</coden><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.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>23500286</pmid><doi>10.1016/j.jacc.2013.01.037</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record>
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issn 0735-1097
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source MEDLINE; Elsevier ScienceDirect Journals; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Adolescent
Age
Age Factors
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Australia
Biological and medical sciences
Blood pressure
Canada
Cardiology
Cardiology. Vascular system
Cardiomyopathy
Cardiomyopathy, Hypertrophic - complications
Cardiomyopathy, Hypertrophic - diagnosis
Cardiomyopathy, Hypertrophic - mortality
Cardiomyopathy, Hypertrophic - therapy
Cardiovascular
Cause of Death
Chi-Square Distribution
Child
Child, Preschool
children
Confidence Intervals
Death, Sudden, Cardiac - etiology
Death, Sudden, Cardiac - prevention & control
Defibrillators
Defibrillators, Implantable - utilization
Disease prevention
Electrocardiography - methods
Emergency and intensive care: neonates and children. Prematurity. Sudden death
Europe
Family medical history
Female
Follow-Up Studies
Heart
Humans
Intensive care medicine
Internal Medicine
Intervention
Kaplan-Meier Estimate
Male
Medical sciences
Myocarditis. Cardiomyopathies
Pediatrics
Proportional Hazards Models
Registries
Risk Assessment
Risk factors
Severity of Illness Index
Sex Factors
sudden death
Survival Analysis
Teenagers
Time Factors
Treatment Outcome
United States
ventricular fibrillation
title Prevention of Sudden Cardiac Death With Implantable Cardioverter-Defibrillators in Children and Adolescents With Hypertrophic Cardiomyopathy
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