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

Journal of Cardiovascular Magnetic Resonance

Research

Renzo Galanello¹  Carlo Dessi¹  Francisco Alpendurada²  Winston Banya²  Mark A Tanner²  John-Paul Carpenter³  Dudley J Pennell³  Gill C Smith³  Sunil V Nair⁴  John Malcolm Walker⁴ 

[1] Ospedale Regionale per le Microcitemie, Cagliari, Italy;Royal Brompton & Harefield NHS Foundation Trust, London, UK;Royal Brompton & Harefield NHS Foundation Trust, London, UK;Imperial College, London, UK;University College Hospital, London, UK;

关键词: thalassaemia major;    deferiprone;    deferoxamine;    right ventricular function;   

DOI  :  10.1186/1532-429X-14-8

 received in 2011-06-28, accepted in 2012-01-25,  发布年份 2012

来源: Springer

【 摘 要 】

BackgroundCombination 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).MethodsWe 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.ResultsIn 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).ConclusionsIn 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.

【 授权许可】

Unknown   
© Alpendurada et al; licensee BioMed Central Ltd. 2012. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.