Registo Português de Miocardiopatia Hipertrófica

Detalhes bibliográficos
Autor(a) principal: Cardim, Nuno
Data de Publicação: 2018
Outros Autores: Brito, Dulce, Rocha Lopes, Luís, Freitas, António, Araújo, Carla, Belo, Adriana, Gonçalves, Lino, Mimoso, Jorge, Olivotto, Iacopo, Elliott, Perry, Madeira, Hugo
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10362/147561
Resumo: Funding Information: Gold: Jaba Recordati, Merck Serono, Sanofi-Genzyme, Shire; Silver: Medinfar, Servier IOi was supported by the Italian Ministry of Health (“LVH in aortic valve disease and HCM: genetic basis, biophysical correlates and viral therapy models” (RF-2013-02356787), and NET-2011-02347173 (“Mechanisms and treatment of coronary microvascular dysfunction in patients with genetic or secondary LVH”); and by Telethon Italy (GGP13162). Publisher Copyright: © 2017 Sociedade Portuguesa de Cardiologia
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spelling Registo Português de Miocardiopatia HipertróficaThe Portuguese Registry of Hypertrophic CardiomyopathyOverall resultsresultados globaisHypertrophic cardiomyopathyLeft ventricular hypertrophyOutcomeRegistryCardiology and Cardiovascular MedicineFunding Information: Gold: Jaba Recordati, Merck Serono, Sanofi-Genzyme, Shire; Silver: Medinfar, Servier IOi was supported by the Italian Ministry of Health (“LVH in aortic valve disease and HCM: genetic basis, biophysical correlates and viral therapy models” (RF-2013-02356787), and NET-2011-02347173 (“Mechanisms and treatment of coronary microvascular dysfunction in patients with genetic or secondary LVH”); and by Telethon Italy (GGP13162). Publisher Copyright: © 2017 Sociedade Portuguesa de CardiologiaIntroduction: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. Methods: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. Results: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. Conclusions: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)RUNCardim, NunoBrito, DulceRocha Lopes, LuísFreitas, AntónioAraújo, CarlaBelo, AdrianaGonçalves, LinoMimoso, JorgeOlivotto, IacopoElliott, PerryMadeira, Hugo2023-01-13T22:20:47Z2018-012018-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article10application/pdfhttp://hdl.handle.net/10362/147561por0870-2551PURE: 50444911https://doi.org/10.1016/j.repc.2017.08.005info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T05:28:47Zoai:run.unl.pt:10362/147561Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:53:00.272853Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Registo Português de Miocardiopatia Hipertrófica
The Portuguese Registry of Hypertrophic CardiomyopathyOverall results
resultados globais
title Registo Português de Miocardiopatia Hipertrófica
spellingShingle Registo Português de Miocardiopatia Hipertrófica
Cardim, Nuno
Hypertrophic cardiomyopathy
Left ventricular hypertrophy
Outcome
Registry
Cardiology and Cardiovascular Medicine
title_short Registo Português de Miocardiopatia Hipertrófica
title_full Registo Português de Miocardiopatia Hipertrófica
title_fullStr Registo Português de Miocardiopatia Hipertrófica
title_full_unstemmed Registo Português de Miocardiopatia Hipertrófica
title_sort Registo Português de Miocardiopatia Hipertrófica
author Cardim, Nuno
author_facet Cardim, Nuno
Brito, Dulce
Rocha Lopes, Luís
Freitas, António
Araújo, Carla
Belo, Adriana
Gonçalves, Lino
Mimoso, Jorge
Olivotto, Iacopo
Elliott, Perry
Madeira, Hugo
author_role author
author2 Brito, Dulce
Rocha Lopes, Luís
Freitas, António
Araújo, Carla
Belo, Adriana
Gonçalves, Lino
Mimoso, Jorge
Olivotto, Iacopo
Elliott, Perry
Madeira, Hugo
author2_role author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
RUN
dc.contributor.author.fl_str_mv Cardim, Nuno
Brito, Dulce
Rocha Lopes, Luís
Freitas, António
Araújo, Carla
Belo, Adriana
Gonçalves, Lino
Mimoso, Jorge
Olivotto, Iacopo
Elliott, Perry
Madeira, Hugo
dc.subject.por.fl_str_mv Hypertrophic cardiomyopathy
Left ventricular hypertrophy
Outcome
Registry
Cardiology and Cardiovascular Medicine
topic Hypertrophic cardiomyopathy
Left ventricular hypertrophy
Outcome
Registry
Cardiology and Cardiovascular Medicine
description Funding Information: Gold: Jaba Recordati, Merck Serono, Sanofi-Genzyme, Shire; Silver: Medinfar, Servier IOi was supported by the Italian Ministry of Health (“LVH in aortic valve disease and HCM: genetic basis, biophysical correlates and viral therapy models” (RF-2013-02356787), and NET-2011-02347173 (“Mechanisms and treatment of coronary microvascular dysfunction in patients with genetic or secondary LVH”); and by Telethon Italy (GGP13162). Publisher Copyright: © 2017 Sociedade Portuguesa de Cardiologia
publishDate 2018
dc.date.none.fl_str_mv 2018-01
2018-01-01T00:00:00Z
2023-01-13T22:20:47Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/147561
url http://hdl.handle.net/10362/147561
dc.language.iso.fl_str_mv por
language por
dc.relation.none.fl_str_mv 0870-2551
PURE: 50444911
https://doi.org/10.1016/j.repc.2017.08.005
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 10
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dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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