Advances in hypertrophic cardiomyopathy

Bibliographic Details
Main Author: Toste, Alexandra
Publication Date: 2022
Format: Other
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10362/143971
Summary: Copyright © 2022 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.
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spelling Advances in hypertrophic cardiomyopathyWhat the cardiologist needs to knowAtrial fibrillationHeart failureHypertrophic cardiomyopathyNonfamilialPrognosisSarcomere positiveSudden cardiac deathTreatmentCopyright © 2022 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.Hypertrophic cardiomyopathy (HCM) is known as the most common genetic heart disease, characterized by otherwise unexplained left ventricular (LV) hypertrophy. In spite of major advances in whole genome sequence techniques, it is still not possible to identify the causal mutation in approximately half of HCM patients. Consequently, a new HCM concept, "beyond the sarcomere" is being developed, supported by data from recent HCM registries which reveal two distinct HCM subgroups: sarcomere positive HCM subgroup and nonfamilial HCM subgroup. Sarcomere positive HCM patients tend to be younger age at diagnosis, have fewer co-morbidities, present more often with reverse septal morphology, more myocardial fibrosis, less LV outflow tract obstruction, and a worse prognosis when compared to nonfamilial HCM patients. These subgroups, with different molecular basis, phenotypes and clinical profiles, will likely require specific management strategies. Important research advances have also been made concerning diagnosis, sudden cardiac death stratification and therapy. In this article, we seek to review recent relevant knowledge, summarizing the advances in this complex and heterogeneous disease.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)RUNToste, Alexandra2022-09-22T22:37:27Z2022-062022-06-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/other11application/pdfhttp://hdl.handle.net/10362/143971eng0304-4750PURE: 46536743https://doi.org/10.1016/j.repc.2021.05.015info:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2024-05-22T18:05:18Zoai:run.unl.pt:10362/143971Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T17:35:52.045697Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Advances in hypertrophic cardiomyopathy
What the cardiologist needs to know
title Advances in hypertrophic cardiomyopathy
spellingShingle Advances in hypertrophic cardiomyopathy
Toste, Alexandra
Atrial fibrillation
Heart failure
Hypertrophic cardiomyopathy
Nonfamilial
Prognosis
Sarcomere positive
Sudden cardiac death
Treatment
title_short Advances in hypertrophic cardiomyopathy
title_full Advances in hypertrophic cardiomyopathy
title_fullStr Advances in hypertrophic cardiomyopathy
title_full_unstemmed Advances in hypertrophic cardiomyopathy
title_sort Advances in hypertrophic cardiomyopathy
author Toste, Alexandra
author_facet Toste, Alexandra
author_role author
dc.contributor.none.fl_str_mv NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
RUN
dc.contributor.author.fl_str_mv Toste, Alexandra
dc.subject.por.fl_str_mv Atrial fibrillation
Heart failure
Hypertrophic cardiomyopathy
Nonfamilial
Prognosis
Sarcomere positive
Sudden cardiac death
Treatment
topic Atrial fibrillation
Heart failure
Hypertrophic cardiomyopathy
Nonfamilial
Prognosis
Sarcomere positive
Sudden cardiac death
Treatment
description Copyright © 2022 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.
publishDate 2022
dc.date.none.fl_str_mv 2022-09-22T22:37:27Z
2022-06
2022-06-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/other
format other
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/143971
url http://hdl.handle.net/10362/143971
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 0304-4750
PURE: 46536743
https://doi.org/10.1016/j.repc.2021.05.015
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv 11
application/pdf
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repository.mail.fl_str_mv info@rcaap.pt
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