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dc.contributor.authorGonzález López, Esther
dc.contributor.authorGagliardi, Christian
dc.contributor.authorDomínguez, Fernando
dc.contributor.authorQuarta, Cristina Candida
dc.contributor.authorDe Haro del Moral, Francisco 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 Pulpón, Luis
dc.contributor.authorRapezzi, Claudio
dc.contributor.authorGarcía Pavía, Pablo
dc.description.abstractAims: Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterised 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. Methods and Results: Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy 12mm, 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. Conclusion: 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.eng
dc.publisherEuropean heart journalspa
dc.rightsAtribución-NoComercial-SinDerivadas 3.0 España*
dc.subjectAmiloidosis cardiacaspa
dc.subjectGammagrafía Tc-DPDspa
dc.titleClinical characteristics of wild-type transthyretin cardiac amyloidosis – Disproving
dc.description.extent833 KBspa

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