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Prognostic significance of disrhythmias in patients with familiar amyloid polyneuropathy

dc.contributor.authorFrancisco, A.R. Gaspar Lopes
dc.contributor.authorDias, N. Cortez
dc.contributor.authorMenezes, M. N.
dc.contributor.authorGuimarães, T.
dc.contributor.authorSilva, G. Lima da
dc.contributor.authorPlácido, R.
dc.contributor.authorMagalhães, A.
dc.contributor.authorInácio, C.
dc.contributor.authorPinto, Fausto J.
dc.contributor.authorCoutinho, C. Azevedo
dc.date.accessioned2018-11-05T11:39:08Z
dc.date.available2018-11-05T11:39:08Z
dc.date.issued2016
dc.description© 2016 European Society of Cardiology. All rights reserved.pt_PT
dc.description.abstractBackground: Rhythm disturbances and conduction defects are common consequences of cardiac amyloid infiltration in familial amyloid polyneuropathy (FAP) V30M-TTR. The occurrence of dysrhythmias appears to increase with the severity of the disease but its prognostic value is still unknown. Purpose: To evaluate the occurrence of arrhythmias according to the patient's age and the duration of symptoms and to analyze its prognostic value. Methods: Prospective observational study of consecutive patients with FAP V30M-TTR who underwent annual cardiac evaluation including Holter recording. Results: During a median follow-up of 55 months, 223 patients were evaluated (44±14 years; 54.3% female) and a total of 777 Holter recordings were performed. It was found that with increasing age, the number of premature supraventricular (Pearson R=0.246, P<0.001, Spearman's Rho=0.246, P<0.001) and ventricular (Pearson R=0.244, P<0.001, Spearman's Rho=0.221, P<0.001) contractions increased, while the mean heart rate decreased (Pearson R=-0.448, P<0.001, Spearman Rho=-0.43, P<0.001). On the other hand, with increasing in symptoms duration, the number of premature ventricular contractions increased significantly (Pearson R=0.144, P=0.001, Spearman's Rho=0.165, P<0.001) (but not supraventricular) and the mean heart rate decreased (Pearson R=-0.153, P=0.001, Spearman's Rho=-0.185, P<0.001). Multivariate Cox regression analysis showed that the independent predictors of mortality were age (HR: 1.073, 95% CI 1.055–1.090), the presence of brady (HR: 1.615, 95% CI 1.071–2.436, P<0.001) or tachyarrhythmias (HR: 1.255, 95% CI 1.255–3.462, P<0.001) and the number of premature supraventricular contractions (HR: 1.439, 95% CI 1.168–1.774, P<0.001). Conclusions: Holter monitoring should be integrated in the periodic evaluation of FAP V30M-TTR patients. The presence of brady or tachyarrhythmias and the number of premature supraventricular contractions are associated with unfavorable prognosis in these patients and should be treated timely.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationEuropean Heart Journal, Volume 37, Issue suppl_1, 1 August 2016, Pages 599–983pt_PT
dc.identifier.doi10.1093/eurheartj/ehw433pt_PT
dc.identifier.issn0195-668X
dc.identifier.urihttp://hdl.handle.net/10451/35268
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.relation.publisherversionhttps://academic.oup.com/eurheartjpt_PT
dc.titlePrognostic significance of disrhythmias in patients with familiar amyloid polyneuropathypt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage684pt_PT
oaire.citation.startPage683pt_PT
oaire.citation.titleEuropean Heart Journalpt_PT
oaire.citation.volume37pt_PT
person.familyNamePinto
person.givenNameFausto J.
person.identifier1308889
person.identifier.ciencia-idC311-AEDD-6DBB
person.identifier.orcid0000-0002-8034-4529
person.identifier.ridG-9363-2015
person.identifier.scopus-author-id7102740158
rcaap.rightsrestrictedAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication5f44176f-69f5-482c-83cd-ab94425a6ec3
relation.isAuthorOfPublication.latestForDiscovery5f44176f-69f5-482c-83cd-ab94425a6ec3

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