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Journal of Cardiovascular Magnetic Resonance
Published in: Journal of Cardiovascular Magnetic Resonance 1/2012

Open Access 01-12-2012 | Research

Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major

Authors: Francisco Alpendurada, Gill C Smith, John-Paul Carpenter, Sunil V Nair, Mark A Tanner, Winston Banya, Carlo Dessi, Renzo Galanello, John Malcolm Walker, Dudley J Pennell

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2012

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Abstract

Background

Combination therapy with deferoxamine and oral deferiprone is superior to deferoxamine alone in removing cardiac iron and improving left ventricular ejection fraction (LVEF). The right ventricle (RV) is also affected by the toxic effects of iron and may cause additional cardiovascular perturbation. We assessed the effects of combination therapy on the RV in thalassaemia major (TM) using cardiovascular magnetic resonance (CMR).

Methods

We retrieved imaging data from 2 treatment trials and re-analyzed the data for the RV responses: Trial 1 was a randomized controlled trial (RCT) of 65 TM patients with mild-moderate cardiac siderosis receiving combination therapy or deferoxamine with placebo; Trial 2 was an open label longitudinal trial assessing combination therapy in 15 TM patients with severe iron loading.

Results

In the RCT, combination therapy with deferoxamine and deferiprone was superior to deferoxamine alone for improving RVEF (3.6 vs 0.7%, p = 0.02). The increase in RVEF was greater with lower baseline T2* 8-12 ms (4.7 vs 0.5%, p = 0.01) than with T2* 12-20 ms (2.2 vs 0.8%, p = 0.47). In patients with severe cardiac siderosis, substantial improvement in RVEF was seen with open-label combination therapy (10.5% ± 5.6%, p < 0.01).

Conclusions

In the RCT of mild to moderate cardiac iron loading, combination treatment improved RV function significantly more than deferoxamine alone. Combination treatment also improved RV function in severe cardiac siderosis. Therefore adding deferiprone to deferoxamine has beneficial effects on both RV and LV function in TM patients with cardiac siderosis.
Appendix
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Metadata
Title
Effects of combined deferiprone with deferoxamine on right ventricular function in thalassaemia major
Authors
Francisco Alpendurada
Gill C Smith
John-Paul Carpenter
Sunil V Nair
Mark A Tanner
Winston Banya
Carlo Dessi
Renzo Galanello
John Malcolm Walker
Dudley J Pennell
Publication date
01-12-2012
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2012
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/1532-429X-14-8

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