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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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 |
format | Article |
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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 & 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>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=27238986$$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 & 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 & 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 & Medical Complete (Alumni)</collection><collection>Nursing & 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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identifier | ISSN: 0735-1097 |
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issn | 0735-1097 1558-3597 |
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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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