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Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies

AIMS: An algorithm for non‐invasive diagnosis of amyloid transthyretin cardiac amyloidosis (ATTR‐CA) and novel disease‐modifying therapies have prompted an active search for CA. We examined the prevalence of CA in different settings based on literature data. METHODS AND RESULTS: We performed a syste...

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Autores principales: Aimo, Alberto, Merlo, Marco, Porcari, Aldostefano, Georgiopoulos, Georgios, Pagura, Linda, Vergaro, Giuseppe, Sinagra, Gianfranco, Emdin, Michele, Rapezzi, Claudio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10084346/
https://www.ncbi.nlm.nih.gov/pubmed/35509173
http://dx.doi.org/10.1002/ejhf.2532
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author Aimo, Alberto
Merlo, Marco
Porcari, Aldostefano
Georgiopoulos, Georgios
Pagura, Linda
Vergaro, Giuseppe
Sinagra, Gianfranco
Emdin, Michele
Rapezzi, Claudio
author_facet Aimo, Alberto
Merlo, Marco
Porcari, Aldostefano
Georgiopoulos, Georgios
Pagura, Linda
Vergaro, Giuseppe
Sinagra, Gianfranco
Emdin, Michele
Rapezzi, Claudio
author_sort Aimo, Alberto
collection PubMed
description AIMS: An algorithm for non‐invasive diagnosis of amyloid transthyretin cardiac amyloidosis (ATTR‐CA) and novel disease‐modifying therapies have prompted an active search for CA. We examined the prevalence of CA in different settings based on literature data. METHODS AND RESULTS: We performed a systematic search for screening studies on CA, focusing on the prevalence, sex and age distribution in different clinical settings. The prevalence of CA in different settings was as follows: bone scintigraphy for non‐cardiac reasons (n = 5 studies), 1% (95% confidence interval [CI] 0%–1%); heart failure with preserved ejection fraction (n = 6), 12% (95% CI 6%–20%); heart failure with reduced or mildly reduced ejection fraction (n = 2), 10% (95% CI 6%–15%); conduction disorders warranting pacemaker implantation (n = 1), 2% (95% CI 0%–4%); surgery for carpal tunnel syndrome (n = 3), 7% (95% CI 5%–10%); hypertrophic cardiomyopathy phenotype (n = 2), 7% (95% CI 5%–9%); severe aortic stenosis (n = 7), 8% (95% CI 5%–13%); autopsy series of ‘unselected’ elderly individuals (n = 4), 21% (95% CI 7%–39%). The average age of CA patients in the different settings ranged from 74 to 90 years, and the percentage of men from 50% to 100%. Many patients had ATTR‐CA, but the average percentage of patients with amyloid light‐chain (AL) CA was up to 18%. CONCLUSIONS: Searching for CA in specific settings allows to identify a relatively high number of cases who may be eligible for treatment if the diagnosis is unequivocal. ATTR‐CA accounts for many cases of CA across the different settings, but AL‐CA is not infrequent. Median age at diagnosis falls in the eighth or ninth decades, and many patients diagnosed with CA are women.
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spelling pubmed-100843462023-04-11 Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies Aimo, Alberto Merlo, Marco Porcari, Aldostefano Georgiopoulos, Georgios Pagura, Linda Vergaro, Giuseppe Sinagra, Gianfranco Emdin, Michele Rapezzi, Claudio Eur J Heart Fail Cardiac Amyloidosis AIMS: An algorithm for non‐invasive diagnosis of amyloid transthyretin cardiac amyloidosis (ATTR‐CA) and novel disease‐modifying therapies have prompted an active search for CA. We examined the prevalence of CA in different settings based on literature data. METHODS AND RESULTS: We performed a systematic search for screening studies on CA, focusing on the prevalence, sex and age distribution in different clinical settings. The prevalence of CA in different settings was as follows: bone scintigraphy for non‐cardiac reasons (n = 5 studies), 1% (95% confidence interval [CI] 0%–1%); heart failure with preserved ejection fraction (n = 6), 12% (95% CI 6%–20%); heart failure with reduced or mildly reduced ejection fraction (n = 2), 10% (95% CI 6%–15%); conduction disorders warranting pacemaker implantation (n = 1), 2% (95% CI 0%–4%); surgery for carpal tunnel syndrome (n = 3), 7% (95% CI 5%–10%); hypertrophic cardiomyopathy phenotype (n = 2), 7% (95% CI 5%–9%); severe aortic stenosis (n = 7), 8% (95% CI 5%–13%); autopsy series of ‘unselected’ elderly individuals (n = 4), 21% (95% CI 7%–39%). The average age of CA patients in the different settings ranged from 74 to 90 years, and the percentage of men from 50% to 100%. Many patients had ATTR‐CA, but the average percentage of patients with amyloid light‐chain (AL) CA was up to 18%. CONCLUSIONS: Searching for CA in specific settings allows to identify a relatively high number of cases who may be eligible for treatment if the diagnosis is unequivocal. ATTR‐CA accounts for many cases of CA across the different settings, but AL‐CA is not infrequent. Median age at diagnosis falls in the eighth or ninth decades, and many patients diagnosed with CA are women. John Wiley & Sons, Ltd. 2022-05-16 2022-12 /pmc/articles/PMC10084346/ /pubmed/35509173 http://dx.doi.org/10.1002/ejhf.2532 Text en © 2022 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Cardiac Amyloidosis
Aimo, Alberto
Merlo, Marco
Porcari, Aldostefano
Georgiopoulos, Georgios
Pagura, Linda
Vergaro, Giuseppe
Sinagra, Gianfranco
Emdin, Michele
Rapezzi, Claudio
Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title_full Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title_fullStr Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title_full_unstemmed Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title_short Redefining the epidemiology of cardiac amyloidosis. A systematic review and meta‐analysis of screening studies
title_sort redefining the epidemiology of cardiac amyloidosis. a systematic review and meta‐analysis of screening studies
topic Cardiac Amyloidosis
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10084346/
https://www.ncbi.nlm.nih.gov/pubmed/35509173
http://dx.doi.org/10.1002/ejhf.2532
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