Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination

Background: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations,...

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Autores principales:	Aldostefano Porcari, Rossana Bussani, Marco Merlo, Guerino Giuseppe Varrà, Linda Pagura, Davide Rozze, Gianfranco Sinagra
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	transthyretin (ATTR) amyloidosis light chain (AL) amyloidosis epidemiology histology red flags amyloidosis Diseases of the circulatory (Cardiovascular) system RC666-701
Acceso en línea:	https://doaj.org/article/53d41203dd8e44d38ab04889748e2522
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spelling	oai:doaj.org-article:53d41203dd8e44d38ab04889748e25222021-11-30T12:15:57ZIncidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination2297-055X10.3389/fcvm.2021.749523https://doaj.org/article/53d41203dd8e44d38ab04889748e25222021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fcvm.2021.749523/fullhttps://doaj.org/toc/2297-055XBackground: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations, and (c) compare their cardiological profiles. After dedicated staining, the localization (interstitial or vascular) and the distribution (non-diffuse or diffuse) of amyloid deposition were analyzed. Cardiological data at last evaluation were retrospectively assessed for the presence of CA red-flags.Results: CA (50% light chains, 50% transthyretin) was found in 43% (n = 24/56) of the autopsied hearts. Atria were involved in 96% of cases. Amyloid localized both at the perivascular and interstitial levels (95.5 and 85%, respectively) with a slightly predominant non-diffuse distribution (58% of cases). Compared to the other patients, CA patients had a more frequent history of heart failure (HF) (79 vs. 47%, p = 0.014), advanced NYHA functional class (III-IV 25 vs. 6%, p = 0.047), atrial fibrillation (68 vs. 36%, p = 0.019), discrepancy between QRS voltage and left ventricular (LV) thickness (70 vs. 12%, p < 0.001), thicker LV walls (15 vs. 11 mm, p < 0.001), enlarged left atrium (49 vs. 42 mm, p = 0.019) and restrictive filling pattern (56 vs. 19%, p = 0.020). The presence of right ventricular amyloidosis seemed to identify hearts with a higher amyloid burden. Among the CA patients, >30% had ≥3 echocardiographic red-flags of disease.Conclusion: CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, LV hypertrophy and atrial fibrillation.Aldostefano PorcariRossana BussaniMarco MerloGuerino Giuseppe VarràLinda PaguraDavide RozzeGianfranco SinagraFrontiers Media S.A.articletransthyretin (ATTR) amyloidosislight chain (AL) amyloidosisepidemiologyhistologyred flagsamyloidosisDiseases of the circulatory (Cardiovascular) systemRC666-701ENFrontiers in Cardiovascular Medicine, Vol 8 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	transthyretin (ATTR) amyloidosis light chain (AL) amyloidosis epidemiology histology red flags amyloidosis Diseases of the circulatory (Cardiovascular) system RC666-701
spellingShingle	transthyretin (ATTR) amyloidosis light chain (AL) amyloidosis epidemiology histology red flags amyloidosis Diseases of the circulatory (Cardiovascular) system RC666-701 Aldostefano Porcari Rossana Bussani Marco Merlo Guerino Giuseppe Varrà Linda Pagura Davide Rozze Gianfranco Sinagra Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
description	Background: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations, and (c) compare their cardiological profiles. After dedicated staining, the localization (interstitial or vascular) and the distribution (non-diffuse or diffuse) of amyloid deposition were analyzed. Cardiological data at last evaluation were retrospectively assessed for the presence of CA red-flags.Results: CA (50% light chains, 50% transthyretin) was found in 43% (n = 24/56) of the autopsied hearts. Atria were involved in 96% of cases. Amyloid localized both at the perivascular and interstitial levels (95.5 and 85%, respectively) with a slightly predominant non-diffuse distribution (58% of cases). Compared to the other patients, CA patients had a more frequent history of heart failure (HF) (79 vs. 47%, p = 0.014), advanced NYHA functional class (III-IV 25 vs. 6%, p = 0.047), atrial fibrillation (68 vs. 36%, p = 0.019), discrepancy between QRS voltage and left ventricular (LV) thickness (70 vs. 12%, p < 0.001), thicker LV walls (15 vs. 11 mm, p < 0.001), enlarged left atrium (49 vs. 42 mm, p = 0.019) and restrictive filling pattern (56 vs. 19%, p = 0.020). The presence of right ventricular amyloidosis seemed to identify hearts with a higher amyloid burden. Among the CA patients, >30% had ≥3 echocardiographic red-flags of disease.Conclusion: CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, LV hypertrophy and atrial fibrillation.
format	article
author	Aldostefano Porcari Rossana Bussani Marco Merlo Guerino Giuseppe Varrà Linda Pagura Davide Rozze Gianfranco Sinagra
author_facet	Aldostefano Porcari Rossana Bussani Marco Merlo Guerino Giuseppe Varrà Linda Pagura Davide Rozze Gianfranco Sinagra
author_sort	Aldostefano Porcari
title	Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
title_short	Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
title_full	Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
title_fullStr	Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
title_full_unstemmed	Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination
title_sort	incidence and characterization of concealed cardiac amyloidosis among unselected elderly patients undergoing post-mortem examination
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/53d41203dd8e44d38ab04889748e2522
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Incidence and Characterization of Concealed Cardiac Amyloidosis Among Unselected Elderly Patients Undergoing Post-mortem Examination

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