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.
To describe 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.
1281 ATTRwt-CA patients were included in the study. 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 NYHA III-IV and chronic kidney disease. Survival was significantly worse in the HF vs. other pathways, but similar among the three others. In multivariate model, older age at diagnosis, NYHA 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. This article is protected by copyright. All rights reserved.
This article is protected by copyright. All rights reserved.
About The Expert
Giacomo Tini
Paolo Milani
Mattia Zampieri
Angelo Giuseppe Caponetti
Francesca 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
References
PubMed