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1. Unmasking the prevalence of amyloid cardiomyopathy in the real world: results from Phase 2 of the AC-TIVE study, an Italian nationwide survey.

Author

Merlo M, Pagura L, Porcari A, Cameli M, Vergaro G, Musumeci B, Biagini E, Canepa M, Crotti L, Imazio M, Forleo C, Cappelli F, Perfetto F, Favale S, Di Bella G, Dore F, Girardi F, Tomasoni D, Pavasini R, Rella V, Palmiero G, Caiazza M, Carella MC, Igoren Guaricci A, Branzi G, Caponetti AG, Saturi G, La Malfa G, Merlo AC, Andreis A, Bruno F, Longo F, Rossi M, Varrà GG, Saro R, Di Ienno L, De Carli G, Giacomin E, Arzilli C, Limongelli G, Autore C, Olivotto I, Badano L, Parati G, Perlini S, Metra M, Emdin M, Rapezzi C, and Sinagra G

Subjects
Humans, Middle Aged, Prevalence, Prospective Studies, Stroke Volume, Ventricular Function, Left, Amyloidosis diagnosis, Cardiomyopathies diagnostic imaging, Cardiomyopathies epidemiology, Heart Failure
Abstract

Aim: To investigate the prevalence of amyloid cardiomyopathy (AC) and the diagnostic accuracy of echocardiographic red flags of AC among consecutive adult patients undergoing transthoracic echocardiogram for reason other than AC in 13 Italian institutions., Methods and Results: This is an Italian prospective multicentre study, involving a clinical and instrumental work-up to assess AC prevalence among patients ≥55 years old with an echocardiogram suggestive of AC (i.e. at least one echocardiographic red flag of AC in hypertrophic, non-dilated left ventricles with preserved ejection fraction). The study was registered at ClinicalTrials.gov (NCT04738266). Overall, 381 patients with an echocardiogram suggestive of AC were identified among a cohort of 5315 screened subjects, and 217 patients completed the investigations. A final diagnosis of AC was made in 62 patients with an estimated prevalence of 29% (95% confidence interval 23%-35%). Transthyretin-related AC (ATTR-AC) was diagnosed in 51 and light chain-related AC (AL-AC) in 11 patients. Either apical sparing or a combination of ≥2 other echocardiographic red flags, excluding interatrial septum thickness, provided a diagnostic accuracy >70%., Conclusion: In a cohort of consecutive adults with echocardiographic findings suggestive of AC and preserved left ventricular ejection fraction, the prevalence of AC (either ATTR or AL) was 29%. Easily available echocardiographic red flags, when combined together, demonstrated good diagnostic accuracy., (© 2022 European Society of Cardiology.)

Published
2022
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2. Unmasking the prevalence of amyloid cardiomyopathy in the real world: results from Phase 2 of the AC-TIVE study, an Italian nationwide survey

Author

Marco Merlo, Linda Pagura, Aldostefano Porcari, Matteo Cameli, Giuseppe Vergaro, Beatrice Musumeci, Elena Biagini, Marco Canepa, Lia Crotti, Massimo Imazio, Cinzia Forleo, Francesco Cappelli, Federico Perfetto, Stefano Favale, Gianluca Di Bella, Franca Dore, Francesca Girardi, Daniela Tomasoni, Rita Pavasini, Valeria Rella, Giuseppe Palmiero, Martina Caiazza, Maria Cristina Carella, Andrea Igoren Guaricci, Giovanna Branzi, Angelo Giuseppe Caponetti, Giulia Saturi, Giovanni La Malfa, Andrea Carlo Merlo, Alessandro Andreis, Francesco Bruno, Francesca Longo, Maddalena Rossi, Guerino Giuseppe Varrà, Riccardo Saro, Luca Di Ienno, Giuseppe De Carli, Elisa Giacomin, Chiara Arzilli, Giuseppe Limongelli, Camillo Autore, Iacopo Olivotto, Luigi Badano, Gianfranco Parati, Stefano Perlini, Marco Metra, Michele Emdin, Claudio Rapezzi, Gianfranco Sinagra, Merlo, Marco, Pagura, Linda, Porcari, Aldostefano, Cameli, Matteo, Vergaro, Giuseppe, Musumeci, Beatrice, Biagini, Elena, Canepa, Marco, Crotti, Lia, Imazio, Massimo, Forleo, Cinzia, Cappelli, Francesco, Perfetto, Federico, Favale, Stefano, Di Bella, Gianluca, Dore, Franca, Girardi, Francesca, Tomasoni, Daniela, Pavasini, Rita, Rella, Valeria, Palmiero, Giuseppe, Caiazza, Martina, Carella, Maria Cristina, Igoren Guaricci, Andrea, Branzi, Giovanna, Caponetti, Angelo Giuseppe, Saturi, Giulia, La Malfa, Giovanni, Merlo, Andrea Carlo, Andreis, Alessandro, Bruno, Francesco, Longo, Francesca, Rossi, Maddalena, Varrà, Guerino Giuseppe, Saro, Riccardo, Di Ienno, Luca, De Carli, Giuseppe, Giacomin, Elisa, Arzilli, Chiara, Limongelli, Giuseppe, Autore, Camillo, Olivotto, Iacopo, Badano, Luigi, Parati, Gianfranco, Perlini, Stefano, Metra, Marco, Emdin, Michele, Rapezzi, Claudio, Sinagra, Gianfranco, Merlo, M, Pagura, L, Porcari, A, Cameli, M, Vergaro, G, Musumeci, B, Biagini, E, Canepa, M, Crotti, L, Imazio, M, Forleo, C, Cappelli, F, Perfetto, F, Favale, S, Di Bella, G, Dore, F, Girardi, F, Tomasoni, D, Pavasini, R, Rella, V, Palmiero, G, Caiazza, M, Carella, M, Igoren Guaricci, A, Branzi, G, Caponetti, A, Saturi, G, La Malfa, G, Merlo, A, Andreis, A, Bruno, F, Longo, F, Rossi, M, Varra, G, Saro, R, Di Ienno, L, De Carli, G, Giacomin, E, Arzilli, C, Limongelli, G, Autore, C, Olivotto, I, Badano, L, Parati, G, Perlini, S, Metra, M, Emdin, M, Rapezzi, C, Sinagra, G, and Michele, Emdin

Subjects
Amyloid cardiomyopathy, Echocardiography, Epidemiology, Light chain amyloidosis, Red flags, Transthyretin amyloidosis, Heart Failure, Left, Stroke Volume, Amyloidosis, Middle Aged, Ventricular Function, Left, Humans, Prevalence, Prospective Studies, Cardiomyopathies, Ventricular Function, Light chain amyloidosi, Red flag, Cardiology and Cardiovascular Medicine
Abstract

Aim: To investigate the prevalence of amyloid cardiomyopathy (AC) and the diagnostic accuracy of echocardiographic red flags of AC among consecutive adult patients undergoing transthoracic echocardiogram for reason other than AC in 13 Italian institutions. Methods and results: This is an Italian prospective multicentre study, involving a clinical and instrumental work-up to assess AC prevalence among patients ≥55 years old with an echocardiogram suggestive of AC (i.e. at least one echocardiographic red flag of AC in hypertrophic, non-dilated left ventricles with preserved ejection fraction). The study was registered at ClinicalTrials.gov (NCT04738266). Overall, 381 patients with an echocardiogram suggestive of AC were identified among a cohort of 5315 screened subjects, and 217 patients completed the investigations. A final diagnosis of AC was made in 62 patients with an estimated prevalence of 29% (95% confidence interval 23%–35%). Transthyretin-related AC (ATTR-AC) was diagnosed in 51 and light chain-related AC (AL-AC) in 11 patients. Either apical sparing or a combination of ≥2 other echocardiographic red flags, excluding interatrial septum thickness, provided a diagnostic accuracy >70%. Conclusion: In a cohort of consecutive adults with echocardiographic findings suggestive of AC and preserved left ventricular ejection fraction, the prevalence of AC (either ATTR or AL) was 29%. Easily available echocardiographic red flags, when combined together, demonstrated good diagnostic accuracy.

Published
2022

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