Diagnostic Pathways to Wild-Type Transthyretin Amyloid Cardiomyopathy: a Multicenter Network Study.

Mar 13, 2023

Experts: 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

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.

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ABOUT THE EXPERTS

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

Giacomo Tini

Cardiology Unit, Ospedale Policlinico San Martino IRCCS, Genoa, Italy.

Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Sant’Andrea Hospital, Rome, Italy.

Paolo Milani

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Mattia Zampieri

Tuscan Regional Amyloidosis Centre|, Careggi University Hospital, Florence, Italy.

Angelo Giuseppe Caponetti

Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Francesca Fabris

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Andrea Foli

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Alessia Argirò

Tuscan Regional Amyloidosis Centre|, Careggi University Hospital, Florence, Italy.

Carlotta Mazzoni

Tuscan Regional Amyloidosis Centre|, Careggi University Hospital, Florence, Italy.

Christian Gagliardi

Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Simone Longhi

Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Giulia Saturi

Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Giuseppe Vergaro

Interdisciplinary Center of Health Sciences, Scuola Superiore Sant’Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

Alberto Aimo

Interdisciplinary Center of Health Sciences, Scuola Superiore Sant’Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

Domitilla Russo

Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Sant’Andrea Hospital, Rome, Italy.

Guerino Giuseppe Varrà

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Matteo Serenelli

Cardiologic Center, University of Ferrara, Ferrara, Italy.

Gioele Fabbri

Cardiologic Center, University of Ferrara, Ferrara, Italy.

Laura De Michieli

Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, Padua, Italy.

Cardiology Unit, University Hospital of Padova, Padua, Italy.

Giuseppe Palmiero

Inherited and Rare Cardiovascular Disease Unit, University of Campania “Luigi Vanvitelli”, AORN dei Colli, Monaldi Hospital, Naples, Italy.

Giuseppe Ciliberti

Cardiology and Arrhythmology Clinic, University Hospital “Lancisi-Umberto I- Salesi”, Ancona, Italy; Department of Biomedical Sciences and Public Health, Marche Polytechnic University, Ancona, Italy.

Samuela Carigi

Cardiology Unit, Infermi Hospital, Rimini, Italy.

Eugenio Sessarego

Cardiology Unit, Ospedale Policlinico San Martino IRCCS, Genoa, Italy.

Giulia Elena Mandoli

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, Siena, Italy.

Giulia Ricci Lucchi

Department of Cardiology, Ospedale per gli Infermi, Faenza, Italy.

Valeria Rella

Cardiology Department, IRCCS Istituto Auxologico Italiano, Milan, Italy; Center for Cardiac Arrhythmias of Genetic Origin, Istituto Auxologico Italiano, IRCCS, Milan, Italy.

Enrico Monti

U.O. Cardiologia, Ospedale di Forlì, AUSL della Romagna, Forlì, Italy.

Elisa Gardini

U.O. Cardiologia, Ospedale di Forlì, AUSL della Romagna, Forlì, Italy.

Michela Bartolotti

U.O. Cardiologia, Ospedale di Cesena, AUSL della Romagna, Cesena, Italy.

Lia Crotti

Cardiology Department, IRCCS Istituto Auxologico Italiano, Milan, Italy; Center for Cardiac Arrhythmias of Genetic Origin, Istituto Auxologico Italiano, IRCCS, Milan, Italy.

Department of Medicine and Surgery, University Milano Bicocca, Milan, Italy.

Elisa Merli

Department of Cardiology, Ospedale per gli Infermi, Faenza, Italy.

Roberta Mussinelli

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Pier Filippo Vianello

Cardiology Unit, Ospedale Policlinico San Martino IRCCS, Genoa, Italy.

Matteo Cameli

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, Siena, Italy.

Francesca Marzo

Cardiology Unit, Infermi Hospital, Rimini, Italy.

Federico Guerra

Cardiology and Arrhythmology Clinic, University Hospital “Lancisi-Umberto I- Salesi”, Ancona, Italy; Department of Biomedical Sciences and Public Health, Marche Polytechnic University, Ancona, Italy.

Giuseppe Limongelli

Inherited and Rare Cardiovascular Disease Unit, University of Campania “Luigi Vanvitelli”, AORN dei Colli, Monaldi Hospital, Naples, Italy.

Alberto Cipriani

Department of Cardio-Thoraco-Vascular Sciences and Public Health, University of Padua, Padua, Italy.

Cardiology Unit, University Hospital of Padova, Padua, Italy.

Stefano Perlini

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Laura Obici

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Federico Perfetto

Tuscan Regional Amyloidosis Centre|, Careggi University Hospital, Florence, Italy.

Camillo Autore

Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Sant’Andrea Hospital, Rome, Italy.

Italo Porto

Cardiology Unit, Ospedale Policlinico San Martino IRCCS, Genoa, Italy.

Department of Internal Medicine, University of Genova, Genoa, Italy.

Claudio Rapezzi

Cardiologic Center, University of Ferrara, Ferrara, Italy.

GVM Care and Research, Maria Cecilia Hospital, Cotignola, Ravenna, Italy.

Gianfranco Sinagra

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Marco Merlo

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.

Beatrice Musumeci

Cardiology, Department of Clinical and Molecular Medicine, Sapienza University of Rome, Sant’Andrea Hospital, Rome, Italy.

Michele Emdin

Interdisciplinary Center of Health Sciences, Scuola Superiore Sant’Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, Italy.

Elena Biagini

Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.

European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart-ERN GUARD-Heart.

Francesco Cappelli

Tuscan Regional Amyloidosis Centre|, Careggi University Hospital, Florence, Italy.

Giovanni Palladini

Department of Molecular Medicine, University of Pavia, Pavia, Italy.

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Pavia, Italy.

Marco Canepa

Cardiology Unit, Ospedale Policlinico San Martino IRCCS, Genoa, Italy.

Department of Internal Medicine, University of Genova, Genoa, Italy.