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.

Amsterdam University Medical Centre Amsterdam the Netherlands

Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital London UK

Centre for Paediatric Inherited and Rare Cardiovascular Disease University College London Institute of Cardiovascular Science London UK

Children's Heart Center 2nd Faculty of Medicine Charles University Prague and Motol University Hospital Prague Czech Republic

Department of Paediatric Cardiology Helsinki University Children's Hospital Helsinki Finland

Department of Pediatric Cardiology Charite Universitatsmedizin Berlin Berlin Germany

Department of Pediatric Cardiology University of Leiden Leiden the Netherlands

Department of Pediatrics Institute of Clinical Sciences Sahlgrenska Academy University of Gothenburg Gothenburg Sweden

Erasmus MC Sophia Children's Hospital Department of Paediatric Cardiology Rotterdam the Netherlands

Hospital Sant Joan de Deu Barcelona Spain

Inherited and Rare Cardiovascular Disease Unit AO dei Colli Monaldi Hospital Universita della Campania Luigi Vanvitelli Naples Italy

The Children's Memorial Health Institute Warsaw Poland

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Analysis of risk stratification and prevention of sudden death in pediatric patients with hypertrophic cardiomyopathy: Dilemmas and clarity