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oai:repisalud.isciii.es:20.500.12105/154082024-09-27T09:18:24Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605

00925njm 22002777a 4500 dc González-López, Esther author Gagliardi, Christian author Dominguez, Fernando author Quarta, Cristina Candida author de Haro-Del Moral, F Javier author Milandri, Agnese author Salas, Clara author Cinelli, Mario author Cobo-Marcos, Marta author Lorenzini, Massimiliano author Lara-Pezzi, Enrique author Foffi, Serena author Alonso-Pulpon, Luis author Rapezzi, Claudio author Garcia-Pavia, Pablo author 2017-06-21 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. Eur Heart J. 2017 Jun 21;38(24):1895-1904 10.1093/eurheartj/ehx043 1522-9645 European heart journal 28329248 http://hdl.handle.net/20.500.12105/15408 Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths.