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Registo Português de Miocardiopatia Hipertrófica

2018-01Artigo científico Acesso aberto
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dc.contributor.authorCardim, Nuno
dc.contributor.authorCardim, Nuno
dc.contributor.authorBrito, Dulce
dc.contributor.authorRocha Lopes, Luís
dc.contributor.authorFreitas, António
dc.contributor.authorAraújo, Carla
dc.contributor.authorBelo, Adriana
dc.contributor.authorGonçalves, Lino
dc.contributor.authorMimoso, Jorge
dc.contributor.authorOlivotto, Iacopo
dc.contributor.authorElliott, Perry
dc.contributor.authorMadeira, Hugo
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.pblSociedade Portuguesa de Cardiologia | Elsevier
dc.date.accessioned2023-01-13T22:20:47Z
dc.date.available2023-01-13T22:20:47Z
dc.date.issued2018-01
dc.descriptionFunding 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
dc.description.abstractIntroduction: 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.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent10
dc.format.extent1121815
dc.identifier.doi10.1016/j.repc.2017.08.005
dc.identifier.issn0870-2551
dc.identifier.otherPURE: 50444911
dc.identifier.otherPURE UUID: d7f96111-2853-40d0-b2bf-8ad30dc44f1c
dc.identifier.otherScopus: 85040629806
dc.identifier.otherPubMed: 29358015
dc.identifier.otherWOS: 000432448500002
dc.identifier.urihttp://hdl.handle.net/10362/147561
dc.identifier.urlhttps://www.scopus.com/pages/publications/85040629806
dc.language.isopor
dc.peerreviewedyes
dc.subjectHypertrophic cardiomyopathy
dc.subjectLeft ventricular hypertrophy
dc.subjectOutcome
dc.subjectRegistry
dc.subjectCardiology and Cardiovascular Medicine
dc.titleRegisto Português de Miocardiopatia Hipertróficapt
dc.title.alternativeThe Portuguese Registry of Hypertrophic CardiomyopathyOverall resultsen
dc.title.subtitleresultados globaispt
dc.typejournal article
degois.publication.firstPage1
degois.publication.issue1
degois.publication.lastPage10
degois.publication.titleRevista Portuguesa de Cardiologia
degois.publication.volume37
dspace.entity.typePublication
person.familyNameCardim
person.givenNameNuno
person.identifier.orcid0000-0002-3812-4872
rcaap.rightsopenAccess
relation.isAuthorOfPublicationbcbf3f33-253a-4b76-a55f-23e4caf28001
relation.isAuthorOfPublication.latestForDiscoverybcbf3f33-253a-4b76-a55f-23e4caf28001

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