Publicação
The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results
| Resumo: | Introduction: 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 diseasespecific treatments that impact the natural history of the disease. |
|---|---|
| Autores principais: | Cardim, Nuno |
| Outros Autores: | Brito, Dulce; Lopes, Luís Rocha; Freitas, António; Araújo, Carla; Belo, Adriana; Gonçalves, Lino; Mimoso, Jorge; Olivotto, Iacopo; Elliott, Perry; Madeira, Hugo |
| Assunto: | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| Ano: | 2018 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Universidade de Lisboa |
| Idioma: | inglês |
| Origem: | Repositório da Universidade de Lisboa |
| _version_ | 1863858382311522304 |
|---|---|
| author | Cardim, Nuno |
| author2 | Brito, Dulce Lopes, Luís Rocha 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 |
| author_facet | Cardim, Nuno Brito, Dulce Lopes, Luís Rocha Freitas, António Araújo, Carla Belo, Adriana Gonçalves, Lino Mimoso, Jorge Olivotto, Iacopo Elliott, Perry Madeira, Hugo |
| author_role | author |
| contributor_name_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| country_str | PT |
| creators_json_str | [{\"Person.name\":\"Cardim, Nuno\"},{\"Person.name\":\"Brito, Dulce\"},{\"Person.name\":\"Lopes, Luís Rocha\"},{\"Person.name\":\"Freitas, António\"},{\"Person.name\":\"Araújo, Carla\"},{\"Person.name\":\"Belo, Adriana\"},{\"Person.name\":\"Gonçalves, Lino\"},{\"Person.name\":\"Mimoso, Jorge\"},{\"Person.name\":\"Olivotto, Iacopo\"},{\"Person.name\":\"Elliott, Perry\"},{\"Person.name\":\"Madeira, Hugo\"}] |
| datacite.contributors.contributor.contributorName.fl_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| datacite.creators.creator.creatorName.fl_str_mv | Cardim, Nuno Brito, Dulce Lopes, Luís Rocha Freitas, António Araújo, Carla Belo, Adriana Gonçalves, Lino Mimoso, Jorge Olivotto, Iacopo Elliott, Perry Madeira, Hugo |
| datacite.date.Accepted.fl_str_mv | 2018-01-01T00:00:00Z |
| datacite.date.available.fl_str_mv | 2018-10-08T13:07:43Z |
| datacite.date.embargoed.fl_str_mv | 2018-10-08T13:07:43Z |
| datacite.rights.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| datacite.subjects.subject.fl_str_mv | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| datacite.titles.title.fl_str_mv | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results Registo Português de Miocardiopatia Hipertrófica : resultados globais |
| dc.contributor.none.fl_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| dc.creator.none.fl_str_mv | Cardim, Nuno Brito, Dulce Lopes, Luís Rocha Freitas, António Araújo, Carla Belo, Adriana Gonçalves, Lino Mimoso, Jorge Olivotto, Iacopo Elliott, Perry Madeira, Hugo |
| dc.date.Accepted.fl_str_mv | 2018-01-01T00:00:00Z |
| dc.date.available.fl_str_mv | 2018-10-08T13:07:43Z |
| dc.date.embargoed.fl_str_mv | 2018-10-08T13:07:43Z |
| dc.format.none.fl_str_mv | application/pdf |
| dc.identifier.none.fl_str_mv | http://hdl.handle.net/10451/34944 |
| dc.language.none.fl_str_mv | eng |
| dc.publisher.none.fl_str_mv | Elsevier |
| dc.rights.cclincense.fl_str_mv | http://creativecommons.org/licenses/by/4.0/ |
| dc.rights.none.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| dc.subject.none.fl_str_mv | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| dc.title.fl_str_mv | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results Registo Português de Miocardiopatia Hipertrófica : resultados globais |
| dc.type.none.fl_str_mv | http://purl.org/coar/resource_type/c_6501 |
| description | Introduction: 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 diseasespecific treatments that impact the natural history of the disease. |
| dirty | 0 |
| eu_rights_str_mv | openAccess |
| format | article |
| fulltext.url.fl_str_mv | https://repositorio.ulisboa.pt/bitstreams/b9b98a40-fb8e-40ee-9947-839d685cecfe/download |
| id | ul_3af0174e1bbddaa3fc0237d4e0a359fb |
| identifier.url.fl_str_mv | http://hdl.handle.net/10451/34944 |
| instacron_str | ul |
| institution | Universidade de Lisboa |
| instname_str | Universidade de Lisboa |
| language | eng |
| network_acronym_str | ul |
| network_name_str | Repositório da Universidade de Lisboa |
| oai_identifier_str | oai:repositorio.ulisboa.pt:10451/34944 |
| organization_str_mv | urn:organizationAcronym:ul |
| person_str_mv | Cardim, Nuno Brito, Dulce Lopes, Luís Rocha Freitas, António Araújo, Carla Belo, Adriana Gonçalves, Lino Mimoso, Jorge Olivotto, Iacopo Elliott, Perry Madeira, Hugo |
| publishDate | 2018 |
| publisher.none.fl_str_mv | Elsevier |
| reponame_str | Repositório da Universidade de Lisboa |
| repository_id_str | urn:repositoryAcronym:ul |
| service_str_mv | urn:repositoryAcronym:ul |
| spelling | engElsevierpt_PTIntroduction: 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 diseasespecific treatments that impact the natural history of the disease.application/pdfpt_PTThe Portuguese Registry of Hypertrophic Cardiomyopathy : overall resultsAlternativeTitlept_PTRegisto Português de Miocardiopatia Hipertrófica : resultados globaisCardim, NunoBrito, DulceLopes, Luís RochaFreitas, AntónioAraújo, CarlaBelo, AdrianaGonçalves, LinoMimoso, JorgeOlivotto, IacopoElliott, PerryMadeira, HugoHostingInstitutionOrganizationalRepositório Científico de Acesso Aberto da ULisboae-mailmailto:repositorio@reitoria.ulisboa.ptrepositorio@reitoria.ulisboa.ptISSNIsPartOf2174-2049DOIIsPartOf10.1016/j.repce.2018.02.0082018-10-08T13:07:43Z20182018-01-01T00:00:00ZHandlehttp://hdl.handle.net/10451/34944http://purl.org/coar/access_right/c_abf2open accessHypertrophic cardiomyopathyRegistryLeft ventricular hypertrophyOutcome796492 bytesliteraturehttp://purl.org/coar/resource_type/c_6501journal article2018http://creativecommons.org/licenses/by/4.0/http://purl.org/coar/access_right/c_abf2application/pdffulltexthttps://repositorio.ulisboa.pt/bitstreams/b9b98a40-fb8e-40ee-9947-839d685cecfe/downloadRevista Portuguesa de Cardiologia (English Edition)371110 |
| spellingShingle | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results Cardim, Nuno Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| subject.fl_str_mv | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| title | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| title_full | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| title_fullStr | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| title_full_unstemmed | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| title_short | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| title_sort | The Portuguese Registry of Hypertrophic Cardiomyopathy : overall results |
| topic | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| topic_facet | Hypertrophic cardiomyopathy Registry Left ventricular hypertrophy Outcome |
| url | http://hdl.handle.net/10451/34944 |
| visible | 1 |