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Title: Cardiovascular magnetic resonance of siderotic cardiomyopathy in thalassaemia major
Author: Tanner, Mark A.
ISNI:       0000 0001 3498 8270
Awarding Body: Imperial College London (University of London)
Current Institution: Imperial College London
Date of Award: 2007
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Background: Heart failure secondary to myocardial iron overload represents the single most important cause of death in thalassaemia major. Cardiovascular magnetic resonance (CMR) has the combmed power to reproducibly assess both myocardial iron loading (T2*) and ventricular ~nctI~n. This makes CMR ideal for assessing new chelation therapies in the treatment of myocardial siderosIs. We aimed to demonstrate the feasibility of inter-scanner transfer of the T2* technique and its use in the assessment ofchelation methods in the context of randomized controlled trials in TM. Methods and Results: We have demonstrated that the CMR T2* technique can be reproducibly transferred to MR scanners in different countries and of different manufacturers with an inter-centre reproducibility of myocardial T2* of 5.0%. We demonstrated a high prevalence of myocardial siderosis in patients maintained on the conventional parenteral chelator, deferoxamine. We have confirmed that the oral chelator deferiprone has superior effects in the removal of myocardial iron and improving ventricular function as compared with deferoxamine. The combination of deferoxamine and deferiprone has significantly greater effects in terms of myocardial siderosis, cardiac function and endothelial function as compared with deferoxamine alone. Furthermore combined chelation therapy is effective in the treatment ofpatients with advanced cardiac siderosis. Conclusions: CMR T2* is a reliable method for the quantification of myocardial iron and can be reproducibly transferred to other MR scanners. Through CMR driven trials we have provided a firm evidence base. for the treatment of myocardial siderosis which should reduce cardiovascular complications and ultimately improve mortality.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available