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Indications and management of implantable cardioverter-defibrillator therapy in childhood hypertrophic cardiomyopathy

Kaski, Juan Pablo; Kammeraad, Janneke AE; Blom, Nico A; Happonen, Juha-Matti; Janousek, Jan; Klaassen, Sabine; Limongelli, Giuseppe; ... Ziolkowska, Lidia; + view all (2023) Indications and management of implantable cardioverter-defibrillator therapy in childhood hypertrophic cardiomyopathy. Cardiology in the Young 10.1017/S1047951123000872. (In press). Green open access

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indications-and-management-of-implantable-cardioverter-defibrillator-therapy-in-childhood-hypertrophic-cardiomyopathy-a-position-statement-from-the-aepc-working-group-on-basic-science-genetics-and-myo.pdf - Published Version

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Abstract

Sudden cardiac death is the most common mode of death during childhood and adolescence in hypertrophic cardiomyopathy, and identifying those individuals at highest risk is a major aspect of clinical care. The mainstay of preventative therapy is the implantable cardioverter-defibrillator, which has been shown to be effective at terminating malignant ventricular arrhythmias in children with hypertrophic cardiomyopathy but can be associated with substantial morbidity. Accurate identification of those children at highest risk who would benefit most from implantable cardioverter-defibrillator implantation while minimising the risk of complications is, therefore, essential. This position statement, on behalf of the Association for European Paediatric and Congenital Cardiology (AEPC), reviews the currently available data on established and proposed risk factors for sudden cardiac death in childhood-onset hypertrophic cardiomyopathy and current approaches for risk stratification in this population. It also provides guidance on identification of individuals at risk of sudden cardiac death and optimal management of implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.

Type: Article
Title: Indications and management of implantable cardioverter-defibrillator therapy in childhood hypertrophic cardiomyopathy
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1017/S1047951123000872
Publisher version: https://doi.org/10.1017/S1047951123000872
Language: English
Additional information: © The Author(s), 2023. Published by Cambridge University Press. This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http:// creativecommons.org/licenses/by/4.0/), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.
Keywords: Hypertrophic cardiomyopathy, child, implantable defibrillator, risk factor, sudden cardiac death
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: https://discovery.ucl.ac.uk/id/eprint/10169485
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