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Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia.

Kirk, P; Sheppard, M; Carpenter, J-P; Anderson, L; He, T; St Pierre, T; Galanello, R; Catani, G; Wood, J; Fucharoen, S; et al. Kirk, P; Sheppard, M; Carpenter, J-P; Anderson, L; He, T; St Pierre, T; Galanello, R; Catani, G; Wood, J; Fucharoen, S; Porter, JB; Walker, JM; Forni, GL; Pennell, DJ (2017) Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia. J Cardiovasc Magn Reson, 19. p. 36. ISSN 1532-429X https://doi.org/10.1186/s12968-017-0349-3
SGUL Authors: He, Taigang Sheppard, Mary Noelle

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Abstract

BACKGROUND: Heart failure related to cardiac siderosis remains a major cause of death in transfusion dependent anaemias. Replacement fibrosis has been reported as causative of heart failure in siderotic cardiomyopathy in historical reports, but these findings do not accord with the reversible nature of siderotic heart failure achievable with intensive iron chelation. METHODS: Ten whole human hearts (9 beta-thalassemia major, 1 sideroblastic anaemia) were examined for iron loading and fibrosis (replacement and interstitial). Five had died from heart failure, 4 had cardiac transplantation for heart failure, and 1 had no heart failure (death from a stroke). Heart samples iron content was measured using atomic emission spectroscopy. Interstitial fibrosis was quantified by computer using picrosirius red (PSR) staining and expressed as collagen volume fraction (CVF) with normal value for left ventricle <3%. RESULTS: The 9 hearts affected by heart failure had severe iron loading with very low T2* of 5.0 ± 2.0 ms (iron concentration 8.5 ± 7.0 mg/g dw) and diffuse granular myocardial iron deposition. In none of the 10 hearts was significant macroscopic replacement fibrosis present. In only 2 hearts was interstitial fibrosis present, but with low CVF: in one patient with no cardiac siderosis (death by stroke, CVF 5.9%) and in a heart failure patient (CVF 2%). In the remaining 8 patients, no interstitial fibrosis was seen despite all having severe cardiac siderosis and heart failure (CVF 1.86% ±0.87%). CONCLUSION: Replacement cardiac fibrosis was not seen in the 9 post-mortem hearts from patients with severe cardiac siderosis and heart failure leading to death or transplantation, which contrasts markedly to historical reports. Minor interstitial fibrosis was also unusual and very limited in extent. These findings accord with the potential for reversibility of heart failure seen in iron overload cardiomyopathy. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT00520559.

Item Type: Article
Additional Information: © The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Keywords: Cardiac MR, Cardiac siderosis, Fibrosis, Heart, Histopathology, Iron, Thalassaemia, Nuclear Medicine & Medical Imaging, 1102 Cardiovascular Medicine And Haematology
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA)
Journal or Publication Title: J Cardiovasc Magn Reson
ISSN: 1532-429X
Language: eng
Dates:
DateEvent
27 March 2017Published
1 March 2017Accepted
Publisher License: Creative Commons: Attribution 4.0
Projects:
Project IDFunderFunder ID
R01 DK066084-02National Institutes of Healthhttp://dx.doi.org/10.13039/100000002
UNSPECIFIEDBritish Heart Foundationhttp://dx.doi.org/10.13039/501100000274
PubMed ID: 28343449
Go to PubMed abstract
URI: http://openaccess.sgul.ac.uk/id/eprint/108751
Publisher's version: https://doi.org/10.1186/s12968-017-0349-3

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