학술논문

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia
Document Type
article
Source
Journal of Cardiovascular Magnetic Resonance, Vol 19, Iss 1, Pp 1-9 (2017)
Subject
Thalassaemia
Cardiac siderosis
Cardiac MR
Iron
Heart
Fibrosis
Diseases of the circulatory (Cardiovascular) system
RC666-701
Language
English
ISSN
1532-429X
Abstract
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