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dc.contributor.author | González-López, Esther | |
dc.contributor.author | Gagliardi, Christian | |
dc.contributor.author | Dominguez, Fernando | |
dc.contributor.author | Quarta, Cristina Candida | |
dc.contributor.author | de Haro-Del Moral, F Javier | |
dc.contributor.author | Milandri, Agnese | |
dc.contributor.author | Salas, Clara | |
dc.contributor.author | Cinelli, Mario | |
dc.contributor.author | Cobo-Marcos, Marta | |
dc.contributor.author | Lorenzini, Massimiliano | |
dc.contributor.author | Lara-Pezzi, Enrique | |
dc.contributor.author | Foffi, Serena | |
dc.contributor.author | Alonso-Pulpon, Luis | |
dc.contributor.author | Rapezzi, Claudio | |
dc.contributor.author | Garcia-Pavia, Pablo | |
dc.date.accessioned | 2023-01-09T13:40:00Z | |
dc.date.available | 2023-01-09T13:40:00Z | |
dc.date.issued | 2017-06-21 | |
dc.identifier.citation | Eur Heart J. 2017 Jun 21;38(24):1895-1904 | es_ES |
dc.identifier.uri | http://hdl.handle.net/20.500.12105/15408 | |
dc.description.abstract | Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy ≥12 mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion). Mutations in TTR were excluded in all cases. The study cohort comprised 108 patients (78.6 ± 8 years); 67 (62%) diagnosed invasively and 41 (38%) non-invasively. Twenty patients (19%) were females. An asymmetric hypertrophy pattern was observed in 25 (23%) patients. Mean LVEF was 52 ± 14%, with 39 patients (37%) showing a LVEF < 50%. Atrial fibrillation (56%) and a pseudo-infarct pattern (63%) were the commonest ECG findings. Only 22 patients fulfilled QRS low-voltage criteria while 10 showed LV hypertrophy on ECG. Although heart failure was the most frequent profile leading to diagnosis (68%), 7% of individuals presented with atrioventricular block and 11% were diagnosed incidentally. Almost one third (35; 32%) were previously misdiagnosed. The clinical spectrum of ATTRwt is heterogeneous and differs from the classic phenotype: women are affected in a significant proportion; asymmetric LV hypertrophy and impaired LVEF are not rare and only a minority have low QRS voltages. Clinicians should be aware of the broad clinical spectrum of ATTRwt to correctly identify an entity for which a number of disease-modifying treatments are under investigation. | es_ES |
dc.description.sponsorship | This work was supported in part by the Spanish Society of Cardiology [Grant 2016 to E.G-L.] and by the Instituto de Salud Carlos III (ISCIII) [grants RD012/0042/0066 and CB16/11/00432] and by the Spanish Ministry of Economy and Competitiveness [grant SAF2015-71863-REDT]. Grants are supported by the Plan Estatal de IþDþI 2013-2016–European Regional Development Fund (FEDER) “A way of making Europe”. | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | Oxford University Press | es_ES |
dc.type.hasVersion | VoR | es_ES |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | * |
dc.subject.mesh | Aged | es_ES |
dc.subject.mesh | Amyloid Neuropathies, Familial | es_ES |
dc.subject.mesh | Cardiomyopathies | es_ES |
dc.subject.mesh | Diagnostic Errors | es_ES |
dc.subject.mesh | Diphosphonates | es_ES |
dc.subject.mesh | Echocardiography | es_ES |
dc.subject.mesh | Electrocardiography | es_ES |
dc.subject.mesh | Female | es_ES |
dc.subject.mesh | Genotyping Techniques | es_ES |
dc.subject.mesh | Humans | es_ES |
dc.subject.mesh | Hypertrophy, Left Ventricular | es_ES |
dc.subject.mesh | Male | es_ES |
dc.subject.mesh | Multimodal Imaging | es_ES |
dc.subject.mesh | Organotechnetium Compounds | es_ES |
dc.subject.mesh | Prospective Studies | es_ES |
dc.subject.mesh | Radiopharmaceuticals | es_ES |
dc.subject.mesh | Single Photon Emission Computed Tomography Computed Tomography | es_ES |
dc.title | Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths. | es_ES |
dc.type | journal article | es_ES |
dc.rights.license | Atribución 4.0 Internacional | * |
dc.identifier.pubmedID | 28329248 | es_ES |
dc.format.volume | 38 | es_ES |
dc.format.number | 24 | es_ES |
dc.format.page | 1895 | es_ES |
dc.identifier.doi | 10.1093/eurheartj/ehx043 | es_ES |
dc.contributor.funder | Sociedad Española de Cardiología | es_ES |
dc.contributor.funder | Instituto de Salud Carlos III | es_ES |
dc.contributor.funder | Ministerio de Economía y Competitividad (España) | es_ES |
dc.contributor.funder | Unión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF) | es_ES |
dc.description.peerreviewed | Sí | es_ES |
dc.identifier.e-issn | 1522-9645 | es_ES |
dc.relation.publisherversion | 10.1093/eurheartj/ehx043 | es_ES |
dc.identifier.journal | European heart journal | es_ES |
dc.repisalud.orgCNIC | CNIC::Grupos de investigación::Regulación Molecular de la Insuficiencia Cardiaca | es_ES |
dc.repisalud.institucion | CNIC | es_ES |
dc.rights.accessRights | open access | es_ES |
dc.relation.projectFECYT | info:eu-repo/grantAgreement/ES/RD012/0042/0066 | es_ES |
dc.relation.projectFECYT | info:eu-repo/grantAgreement/ES/CB16/11/00432 | es_ES |
dc.relation.projectFECYT | info:eu-repo/grantAgreement/ES/SAF2015-71863-REDT | es_ES |