biostudies-literatureNorrish GCareer Development FellowshipUniversity College London HospitalsBritish Heart FoundationUK Department of Health’sMedical Research Council ClinicalUniversity College LondonNHMRCNational Institutes of HealthClinical Academic Research PartnershipMyokardia, IncGreat Ormond Street Hospital Childrens CharityMax’s Foundation and Great Ormond Street Hospital Children’s CharityMedical Research CouncilNational Institute for Health Research Biomedical Research CentresNational Institute for Health Research (NIHR)National Health and Medical Research Council678-686https://www.ebi.ac.uk/biostudies/studies/S-EPMC8967478biostudies-literatureUnknown29(4)<h4>Aims</h4>Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM). The newly developed HCM Risk-Kids model provides clinicians with individualized estimates of risk. The aim of this study was to externally validate the model in a large independent, multi-centre patient cohort.<h4>Methods and results</h4>A retrospective, longitudinal cohort of 421 patients diagnosed with HCM aged 1-16 years independent of the HCM Risk-Kids development and internal validation cohort was studied. Data on HCM Risk-Kids predictor variables (unexplained syncope, non-sustained ventricular tachycardia, maximal left ventricular wall thickness, left atrial diameter, and left ventricular outflow tract gradient) were collected from the time of baseline clinical evaEuropean journal of preventive cardiologyExternal validation of the HCM Risk-Kids model for predicting sudden cardiac death in childhood hypertrophic cardiomyopathy.PMC8967478NIHR200158FS/16/72/32270W1135#1162929#1154992MR/T024062/1Qu CKhraiche DYamazawa HIngles JHelms AColan SElliott PMOjala THOmar RZCervi EMarrone CSchouvey SField ECenteno FSaberi SHo CYDay SMSinagra GNorrish GRossano JKaski JPKlaassen SRussell MSemsarian CPopoiu AOlivotto IWittekind SGfalseExternal validation of the HCM Risk-Kids model for predicting sudden cardiac death in childhood hypertrophic cardiomyopathy.<h4>Aims</h4>Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM). The newly developed HCM Risk-Kids model provides clinicians with individualized estimates of risk. The aim of this study was to externally validate the model in a large independent, multi-centre patient cohort.<h4>Methods and results</h4>A retrospective, longitudinal cohort of 421 patients diagnosed with HCM aged 1-16 years independent of the HCM Risk-Kids development and internal validation cohort was studied. Data on HCM Risk-Kids predictor variables (unexplained syncope, non-sustained ventricular tachycardia, maximal left ventricular wall thickness, left atrial diameter, and left ventricular outflow tract gradient) were collected from the time of baseline clinical eva2022-01-01T00:00:00Z2022 Mar2026-06-07T03:22:54.75Z2025-04-19T12:55:44.473ZS-EPMC89674783471852810.1093/eurjpc/zwab181