Temporal implementation of a regional referral pathway in transthyretin cardiac amyloidosis: Emilia-Romagna experience
Year: 2024
Type: article
Study design: Observational Study
Abstract: AIMS: Transthyretin cardiac amyloidosis (ATTR-CA) is a rare and progressive cardiomyopathy caused by amyloid fibril deposition in myocardial tissue. Diagnostic challenges have historically hampered timely detection. Recent advances in noninvasive diagnostic techniques have facilitated ATTR-CA diagnosis. We aimed to examine the development of a regional network for the diagnosis and management of ATTR-CA and describe a cohort of patients with ATTR-CA, investigate diagnostic pathways and assess clinical outcomes according to diagnosis periods. METHODS: We performed a survey study analyzing answers from 11 cardiology centers and we conducted a retrospective study including patients with ATTR-CA attending a referral center between 1 January 2012 and 31 December 2022, and categorized by the period of diagnosis (2012-2016 and 2017-2022). RESULTS: Over the years, a growing number of patients reached a diagnosis and were treated in the surveyed nonreferral centers of the region. The retrospective study showed a more significant diagnostic delay in the earlier period rather than the later one [13.4 (5-30.2) vs. 10.6 (5.0-17.9) months, P = 0.04]. Patients diagnosed after 2017 showed a greater survival rate than those diagnosed earlier ( P = 0.02). In the multivariate analysis, the year of diagnosis from 2017 remained independently associated with mortality [hazard ratio (HR) 0.46, 95% confidence interval (CI) 0.28-0.79; P = 0.005]. CONCLUSION: This study emphasized the shift toward noninvasive diagnostic criteria. It revealed a positive impact on patient survival and disease management with the use of disease-modifying therapies and diagnostic developments in more recent years. The findings underscore the importance of disease awareness and networking to reduce diagnostic delays and enhance patient journeys for ATTR-CA.
Authors: Simone Longhi, Elena Biagini, Pietro Guaraldi, Samuela Carigi, Marco Currò Dossi, Michela Bartolotti, Elisa Gardini, Elisa Merli, Francesca Marzo, Giovanni Andrea Luisi, Emanuela Postiglione, Matteo Serenelli, Valeria Tugnoli, Riccardo De Gennaro, Angelo Giuseppe Caponetti, Christian Gagliardi, Giulia Saturi, Alberto Ponziani, Enrica Perugini, Rita Rinaldi, Andrea Barbieri, Silvia Bonatti, Alessandra Ariatti, Chiara Leuzzi, Luca Codeluppi, Walter Serra, Isabella Allegri, Gianluca Lanati, Chiara Terracciano, Pietro Cortelli, Nazzareno Galiè, Giuseppe Boriani
Institutions: IRCCS Azienda Ospedliero-Universitaria di Bologna Policlinico di Sant'Orsola, ERN GUARD-Heart, University of Bologna, Istituto delle Scienze Neurologiche di Bologna, Azienda Unità Sanitaria Locale Della Romagna, Ospedale Infermi di Rimini, Ospedale “M. Bufalini” di Cesena, Ospedale G.B. Morgagni - L.Pierantoni, Ospedale per gli Infermi, Ospedale "Santa Maria delle Croci" di Ravenna, Arcispedale Sant'Anna, Ospedale Maggiore Carlo Alberto Pizzardi, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria di Modena, Azienda Sanitaria Unità Locale di Reggio Emilia, University of Parma, Ospedale di Castel San Giovanni, Guglielmo da Saliceto Hospital
Language: English
Subfield: Internal Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W4401170331