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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorNorrish, Gabrielle
dc.contributor.authorDing, Tao
dc.contributor.authorField, Ella
dc.contributor.authorCervi, Elena
dc.contributor.authorZiolkowska, Lidia
dc.contributor.authorOlivotto, Iacopo
dc.contributor.authorGran Ipiña, Ferran
dc.date.accessioned2022-09-09T12:46:48Z
dc.date.available2022-09-09T12:46:48Z
dc.date.issued2022-05
dc.identifier.citationNorrish G, Ding T, Field E, Cervi E, Ziółkowska L, Olivotto I, et al. Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy. Circ Arrhythmia Electrophysiol. 2022 May;15(5):e010075.
dc.identifier.issn1941-3084
dc.identifier.urihttp://hdl.handle.net/11351/8118
dc.descriptionChild; Death; Hypertrophic cardiomyopathy
dc.description.abstractBackground: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort. Methods: The study cohort comprised 1075 children (mean age, 10.2 years [±4.4]) diagnosed with HCM (1–16 years) from the International Paediatric Hypertrophic Cardiomyopathy Consortium. Anonymized, noninvasive clinical data were collected from baseline evaluation and follow-up, and 5-year estimated SCD risk was calculated (HCM Risk-Kids). Results: MLVWT Z score was <10 in 598 (58.1%), ≥10 to <20 in 334 (31.1%), and ≥20 in 143 (13.3%). Higher MLVWT Z scores were associated with heart failure symptoms, unexplained syncope, left ventricular outflow tract obstruction, left atrial dilatation, and nonsustained ventricular tachycardia. One hundred twenty-two patients (71.3%) with MLVWT Z score ≥20 had coexisting risk factors for SCD. Over a median follow-up of 4.9 years (interquartile range, 2.3–9.3), 115 (10.7%) had an SCD event. Freedom from SCD event at 5 years for those with MLVWT Z scores <10, ≥10 to <20, and ≥20 was 95.6%, 87.4%, and 86.0, respectively. The estimated SCD risk at 5 years had a nonlinear, inverted U-shaped relationship with MLVWT Z score, peaking at Z score +23. The presence of coexisting risk factors had a summative effect on risk. Conclusions: In children with HCM, an inverted U-shaped relationship exists between left ventricular hypertrophy and estimated SCD risk. The presence of additional risk factors has a summative effect on risk. While MLVWT is important for risk stratification, it should not be used either as a binary variable or in isolation to guide implantable cardioverter defibrillator implantation decisions in children with HCM.
dc.language.isoeng
dc.publisherLippincott Williams & Wilkins
dc.relation.ispartofseriesCirculation: Arrhythmia and Electrophysiology;15(5)
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectCor - Hipertròfia - Complicacions
dc.subjectMort sobtada
dc.subjectCor - Ventricle esquerre
dc.subject.meshCardiomyopathy, Hypertrophic
dc.subject.meshDeath, Sudden, Cardiac
dc.subject.meshHeart Ventricles
dc.titleRelationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1161/CIRCEP.121.010075
dc.subject.decsmiocardiopatía hipertrófica
dc.subject.decsmuerte súbita cardíaca
dc.subject.decsventrículos cardíacos
dc.relation.publishversionhttps://doi.org/10.1161/CIRCEP.121.010075
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Norrish G] Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom. Institute of Cardiovascular Sciences, University College London, United Kingdom. [Ding T] Department of Statistical Science, University College London, United Kingdom. [Field E, Cervi E] Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom. [Ziółkowska L] The Children’s Memorial Health Institute, Warsaw, Poland. [Olivotto I] Careggi University Hopsital, Florence, Italy. [Gran F] Vall d’Hebron Hospital Universitari, Barcelona, Spain
dc.identifier.pmid35491873
dc.identifier.wos000796080400004
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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