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Diagnostic pathways to wild‐type transthyretin amyloid cardiomyopathy: a multicentre network study

2023; Elsevier BV; Volume: 25; Issue: 6 Linguagem: Inglês

10.1002/ejhf.2823

1879-0844

Giacomo Tini, Paolo Milani, Mattia Zampieri, Angelo Giuseppe Caponetti, F Fabris, Andrea Foli, Alessia Argirò, Carlotta Mazzoni, Christian Gagliardi, Simone Longhi, Giulia Saturi, Giuseppe Vergaro, Alberto Aimo, Domitilla Russo, Guerino Giuseppe Varrà, Matteo Serenelli, Gioele Fabbri, Laura De Michieli, Giuseppe Palmiero, Giuseppe Ciliberti, Samuela Carigi, Eugenio Sessarego, Giulia Elena Mandoli, Giulia Ricci Lucchi, Valeria Rella, Enrico Monti, Elisa Gardini, Michela Bartolotti, Lia Crotti, Elisa Merli, Roberta Mussinelli, Pier Filippo Vianello, Matteo Cameli, Francesca Marzo, Federico Guerra, Giuseppe Limongelli, Alberto Cipriani, Stefano Perlini, Laura Obici, Federico Perfetto, Camillo Autore, Italo Porto, Claudio Rapezzi, Gianfranco Sinagra, Marco Merlo, Beatrice Musumeci, Michele Emdin, Elena Biagini, Francesco Cappelli, Giovanni Palladini, Marco Canepa,

Cellular transport and secretion

Epidemiology of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) remains poorly defined. A better characterization of pathways leading to ATTRwt-CA diagnosis is of key importance, and potentially informative of disease course and prognosis. The aim of this study was to describe the characteristics of contemporary pathways leading to ATTRwt-CA diagnosis, and their potential association with survival.This was a retrospective study of patients diagnosed with ATTRwt-CA at 17 Italian referral centres for CA. Patients were categorized into different 'pathways' according to the medical reason that triggered the diagnosis of ATTRwt-CA (hypertrophic cardiomyopathy [HCM] pathway, heart failure [HF] pathway, incidental imaging or incidental clinical pathway). Prognosis was investigated with all-cause mortality as endpoint. Overall, 1281 ATTRwt-CA patients were included in the study. The diagnostic pathway leading to ATTRwt-CA diagnosis was HCM in 7% of patients, HF in 51%, incidental imaging in 23%, incidental clinical in 19%. Patients in the HF pathway, as compared to the others, were older and had a greater prevalence of New York Heart Association (NYHA) class III-IV and chronic kidney disease. Survival was significantly worse in the HF versus other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA class III-IV and some comorbidities but not the HF pathway were independently associated with worse survival.Half of contemporary ATTRwt-CA diagnoses occur in a HF setting. These patients had worse clinical profile and outcome than those diagnosed either due to suspected HCM or incidentally, although prognosis remained primarily related to age, NYHA functional class and comorbidities rather than the diagnostic pathway itself.