Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy

Childhood hypertrophic cardiomyopathy; Outcomes; Phenotype

Detalles Bibliográficos
Autores: Norrish, Gabrielle, Cleary, Aoife, Field, Ella, Boleti, Olga, Ziolkowska, Lidia, Gran Ipiña, Ferran, Cervi, Elena
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:11351/8165
Acceso en línea:https://hdl.handle.net/11351/8165
http://hdl.handle.net/11351/8165
Access Level:acceso abierto
Palabra clave:Miocardi - Malalties - Diagnòstic
Cor - Hipertròfia - Diagnòstic
Insuficiència cardíaca
DISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies::Cardiomyopathy, Hypertrophic
Other subheadings::Other subheadings::/diagnosis
DISEASES::Cardiovascular Diseases::Heart Diseases::Heart Failure
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Procedures::Heart Transplantation
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías::miocardiopatía hipertrófica
Otros calificadores::Otros calificadores::/diagnóstico
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::insuficiencia cardíaca
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos cardiovasculares::procedimientos quirúrgicos cardíacos::trasplante de corazón
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oai_identifier_str oai:recercat.cat:11351/8165
network_acronym_str ES
network_name_str España
repository_id_str
dc.title.none.fl_str_mv Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
title Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
spellingShingle Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
Norrish, Gabrielle
Miocardi - Malalties - Diagnòstic
Cor - Hipertròfia - Diagnòstic
Insuficiència cardíaca
DISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies::Cardiomyopathy, Hypertrophic
Other subheadings::Other subheadings::/diagnosis
DISEASES::Cardiovascular Diseases::Heart Diseases::Heart Failure
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Procedures::Heart Transplantation
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías::miocardiopatía hipertrófica
Otros calificadores::Otros calificadores::/diagnóstico
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::insuficiencia cardíaca
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos cardiovasculares::procedimientos quirúrgicos cardíacos::trasplante de corazón
title_short Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
title_full Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
title_fullStr Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
title_full_unstemmed Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
title_sort Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy
dc.creator.none.fl_str_mv Norrish, Gabrielle
Cleary, Aoife
Field, Ella
Boleti, Olga
Ziolkowska, Lidia
Gran Ipiña, Ferran
Cervi, Elena
author Norrish, Gabrielle
author_facet Norrish, Gabrielle
Cleary, Aoife
Field, Ella
Boleti, Olga
Ziolkowska, Lidia
Gran Ipiña, Ferran
Cervi, Elena
author_role author
author2 Cleary, Aoife
Field, Ella
Boleti, Olga
Ziolkowska, Lidia
Gran Ipiña, Ferran
Cervi, Elena
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Institut Català de la Salut
[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
Vall d'Hebron Barcelona Hospital Campus
dc.subject.none.fl_str_mv Miocardi - Malalties - Diagnòstic
Cor - Hipertròfia - Diagnòstic
Insuficiència cardíaca
DISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies::Cardiomyopathy, Hypertrophic
Other subheadings::Other subheadings::/diagnosis
DISEASES::Cardiovascular Diseases::Heart Diseases::Heart Failure
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Procedures::Heart Transplantation
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías::miocardiopatía hipertrófica
Otros calificadores::Otros calificadores::/diagnóstico
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::insuficiencia cardíaca
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos cardiovasculares::procedimientos quirúrgicos cardíacos::trasplante de corazón
topic Miocardi - Malalties - Diagnòstic
Cor - Hipertròfia - Diagnòstic
Insuficiència cardíaca
DISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies::Cardiomyopathy, Hypertrophic
Other subheadings::Other subheadings::/diagnosis
DISEASES::Cardiovascular Diseases::Heart Diseases::Heart Failure
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Procedures::Heart Transplantation
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías::miocardiopatía hipertrófica
Otros calificadores::Otros calificadores::/diagnóstico
ENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::insuficiencia cardíaca
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos cardiovasculares::procedimientos quirúrgicos cardíacos::trasplante de corazón
description Childhood hypertrophic cardiomyopathy; Outcomes; Phenotype
publishDate 2022
dc.date.none.fl_str_mv 2022
2022
2022
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/11351/8165
http://hdl.handle.net/11351/8165
url https://hdl.handle.net/11351/8165
http://hdl.handle.net/11351/8165
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Journal of the American College of Cardiology;79(20)
https://doi.org/10.1016/j.jacc.2022.03.347
dc.rights.none.fl_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv Scientia
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
repository.name.fl_str_mv
repository.mail.fl_str_mv
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spelling Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic CardiomyopathyNorrish, GabrielleCleary, AoifeField, EllaBoleti, OlgaZiolkowska, LidiaGran Ipiña, FerranCervi, ElenaMiocardi - Malalties - DiagnòsticCor - Hipertròfia - DiagnòsticInsuficiència cardíacaDISEASES::Cardiovascular Diseases::Heart Diseases::Cardiomyopathies::Cardiomyopathy, HypertrophicOther subheadings::Other subheadings::/diagnosisDISEASES::Cardiovascular Diseases::Heart Diseases::Heart FailureANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Surgical Procedures, Operative::Cardiovascular Surgical Procedures::Cardiac Surgical Procedures::Heart TransplantationENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::miocardiopatías::miocardiopatía hipertróficaOtros calificadores::Otros calificadores::/diagnósticoENFERMEDADES::enfermedades cardiovasculares::enfermedades cardíacas::insuficiencia cardíacaTÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::intervenciones quirúrgicas::procedimientos quirúrgicos cardiovasculares::procedimientos quirúrgicos cardíacos::trasplante de corazónChildhood hypertrophic cardiomyopathy; Outcomes; PhenotypeMiocardiopatía hipertrófica infantil; Resultados; FenotipoMiocardiopatia hipertròfica infantil; Resultats; FenotipBackground 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.This work was supported by the British Heart Foundation (grant FS/16/72/32270) to Drs Norrish and Kaski. This work is (partly) funded by the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. Dr Norrish is supported by Great Ormond Street Hospital Children’s Charity. Drs Field and Kaski are supported by Max’s Foundation and Great Ormond Street Hospital Children’s Charity. Dr Kaski is supported by a Medical Research Council–National Institute for Health Research Clinical Academic Research Partnership award. This work was financially supported by the Foundation for Paediatric Research of Finland (Dr Ojala). Dr Fernandez has received speaker fees from Sanofi-Genzyme. Dr Kubus is supported by MH CZ – DRO, Motol University Hospital (00064203). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.ElsevierInstitut Català de la Salut[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, SpainVall d'Hebron Barcelona Hospital Campus202220222022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/11351/8165http://hdl.handle.net/11351/8165Scientiareponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésJournal of the American College of Cardiology;79(20)https://doi.org/10.1016/j.jacc.2022.03.347Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:recercat.cat:11351/81652026-05-29T05:05:01Z
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