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dc.contributor.authorGonzález-López, Esther
dc.contributor.authorGagliardi, Christian
dc.contributor.authorDominguez, Fernando 
dc.contributor.authorQuarta, Cristina Candida
dc.contributor.authorde Haro-Del Moral, F Javier
dc.contributor.authorMilandri, Agnese
dc.contributor.authorSalas, Clara
dc.contributor.authorCinelli, Mario
dc.contributor.authorCobo-Marcos, Marta
dc.contributor.authorLorenzini, Massimiliano
dc.contributor.authorLara-Pezzi, Enrique 
dc.contributor.authorFoffi, Serena
dc.contributor.authorAlonso-Pulpon, Luis
dc.contributor.authorRapezzi, Claudio
dc.contributor.authorGarcia-Pavia, Pablo
dc.identifier.citationEur Heart J. 2017 Jun 21;38(24):1895-1904es_ES
dc.description.abstractWild-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.sponsorshipThis 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.publisherOxford University Press es_ES
dc.subject.meshAged es_ES
dc.subject.meshAmyloid Neuropathies, Familial es_ES
dc.subject.meshCardiomyopathies es_ES
dc.subject.meshDiagnostic Errors es_ES
dc.subject.meshDiphosphonates es_ES
dc.subject.meshEchocardiography es_ES
dc.subject.meshElectrocardiography es_ES
dc.subject.meshFemale es_ES
dc.subject.meshGenotyping Techniques es_ES
dc.subject.meshHumans es_ES
dc.subject.meshHypertrophy, Left Ventricular es_ES
dc.subject.meshMale es_ES
dc.subject.meshMultimodal Imaging es_ES
dc.subject.meshOrganotechnetium Compounds es_ES
dc.subject.meshProspective Studies es_ES
dc.subject.meshRadiopharmaceuticals es_ES
dc.subject.meshSingle Photon Emission Computed Tomography Computed Tomography es_ES
dc.titleClinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths.es_ES
dc.typejournal articlees_ES
dc.rights.licenseAtribución 4.0 Internacional*
dc.contributor.funderSociedad Española de Cardiología es_ES
dc.contributor.funderInstituto de Salud Carlos III es_ES
dc.contributor.funderMinisterio de Economía y Competitividad (España) es_ES
dc.contributor.funderUnión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF) es_ES
dc.identifier.journalEuropean heart journales_ES
dc.repisalud.orgCNICCNIC::Grupos de investigación::Regulación Molecular de la Insuficiencia Cardiacaes_ES
dc.rights.accessRightsopen accesses_ES

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