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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorNorrish, Gabrielle
dc.contributor.authorCleary, Aoife
dc.contributor.authorField, Ella
dc.contributor.authorCervi, Elena
dc.contributor.authorBoleti, Olga
dc.contributor.authorZiolkowska, Lidia
dc.contributor.authorGran Ipiña, Ferran
dc.date.accessioned2022-09-12T10:21:31Z
dc.date.available2022-09-12T10:21:31Z
dc.date.issued2022-05-24
dc.identifier.citationNorrish G, Cleary A, Field E, Cervi E, Boleti O, Ziółkowska L, et al. Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy. J Am Coll Cardiol. 2022 May 24;79(20):1986–97.
dc.identifier.issn0735-1097
dc.identifier.urihttp://hdl.handle.net/11351/8165
dc.descriptionChildhood hypertrophic cardiomyopathy; Outcomes; Phenotype
dc.description.abstractBackground Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized. Objectives The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years. Methods Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years. Results At baseline, 339 patients (53.6%) had family histories of HCM, 132 (20.9%) had heart failure symptoms, and 250 (39.2%) were prescribed cardiac medications. The median maximal left ventricular wall thickness z-score was 8.7 (IQR: 5.3-14.4), and 145 patients (27.2%) had left ventricular outflow tract obstruction. Over a median follow-up period of 5.6 years (IQR: 2.3-10.0 years), 42 patients (6.6%) died, 21 (3.3%) underwent cardiac transplantation, and 69 (10.8%) had life-threatening arrhythmic events. Compared with those presenting after 12 years, a higher proportion of younger patients underwent myectomy (10.5% vs 7.2%; P = 0.045), but fewer received primary prevention implantable cardioverter-defibrillators (18.9% vs 30.1%; P = 0.041). The incidence of mortality or life-threatening arrhythmic events did not differ, but events occurred at a younger age. Conclusions Early-onset childhood HCM is associated with a comparable symptom burden and cardiac phenotype as in patients presenting later in childhood. Long-term outcomes including mortality did not differ by age of presentation, but patients presenting at younger than 12 years experienced adverse events at younger ages.
dc.language.isoeng
dc.publisherElsevier
dc.relation.ispartofseriesJournal of the American College of Cardiology;79(20)
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectMiocardi - Malalties - Diagnòstic
dc.subjectCor - Hipertròfia - Diagnòstic
dc.subjectInsuficiència cardíaca
dc.subject.meshCardiomyopathy, Hypertrophic
dc.subject.mesh/diagnosis
dc.subject.meshHeart Failure
dc.subject.meshHeart Transplantation
dc.titleClinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1016/j.jacc.2022.03.347
dc.subject.decsmiocardiopatía hipertrófica
dc.subject.decs/diagnóstico
dc.subject.decsinsuficiencia cardíaca
dc.subject.decstrasplante de corazón
dc.relation.publishversionhttps://doi.org/10.1016/j.jacc.2022.03.347
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, London, United Kingdom. [Cleary A, Field E, Cervi E] Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom. [Boleti O] Institute of Cardiovascular Sciences, University College London, London, United Kingdom. [Ziółkowska L] The Children’s Memorial Health Institute, Warsaw, Poland. [Gran F] Vall d’Hebron Hospital Universitari, Barcelona, Spain
dc.identifier.pmid35589160
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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