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Title: Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia
Authors: Paul Kirk
Mary Sheppard
John Paul Carpenter
Lisa Anderson
Taigang He
Tim St Pierre
Renzo Galanello
Gualtiero Catani
John Wood
Suthat Fucharoen
John B. Porter
J. Malcolm Walker
Gian Luca Forni
Dudley J. Pennell
Royal Brompton Hospital
National Heart and Lung Institute
University of Western Australia
Azienda Ospedaliera Brotzu - Microcitemico
Children's Hospital Los Angeles
Mahidol University
E.O. Ospedali Galliera
Keywords: Health Professions
Issue Date: 27-Mar-2017
Citation: Journal of Cardiovascular Magnetic Resonance. Vol.19, No.1 (2017)
Abstract: © 2017 The Author(s). 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: Identifier: NCT00520559
ISSN: 1532429X
Appears in Collections:Scopus 2016-2017

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