Effect of deferiprone or deferoxamine on right ventricular function in thalassemia major patients with myocardial iron overload

Journal of Cardiovascular Magnetic Resonance

Research

Francisco Alpendurada¹  Gillian C Smith²  John Paul Carpenter²  Dudley J Pennell²  Mohammed H Alam²  Renzo Galanello³  Mark A Tanner⁴  Mark A Westwood⁵  Efstathios D Gotsis⁶  Vasili Berdoukas⁷  Antonio Piga⁸  Athanassios Aessopos⁹  Markissia Karagiorga^1,10  Vasili Ladis^1,10  Michael Roughton^1,11 

[1] CMR Unit, Brompton & Harefield NHS Foundation Trust, Sydney Street, SW3 6NP, London, UK;CMR Unit, Brompton & Harefield NHS Foundation Trust, Sydney Street, SW3 6NP, London, UK;National Heart & Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, SW3 6LY, London, UK;Department of Biomedical Science and Biotechnology, University of Cagliari, Ospedale Regionale per le Microcitemie. Via Edward Jenner, 09121, Cagliari, Italy;Department of Cardiology, St Richard's Hospital, Western Sussex Hospitals NHS Trust, PO19 6SE, Chichester, West Sussex, UK;Department of Cardiology, The London Chest Hospital, Bonner Road, E2 9JX, London, UK;Department of Magnetic Resonance, Institute Euromedica-Encephalos, 3 Rizariou Street, 152 33, Halandri, Athens, Greece;Division of Haematology/Oncology, Children's Hospital, 4650 Sunset Boulevard, 90027, Los Angeles, CA, USA;Division of Paediatrics and thalassaemia centre, Department of Clinical and Biological Sciences, University of Torino, S. Luigi Gonzaga Hospital, Regione Gonzole 10, 10043, Orbassano, Torino, Italy;First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, 17 Agiou Thoma Street, 115 27, Athens, Greece;Thalassaemia Unit, Aghia Sophia Children's Hospital, Thivon & Papadiamantopoulou, 115 27, Goudi, Athens, Greece;UCL Cancer Trials Centre, University College London, 90 Tottenham Court Road, W1T 4TJ, London, UK;

关键词: Cardiovascular Magnetic Resonance;    Right Ventricle;    Deferoxamine;    Deferasirox;    Deferiprone;   

DOI  :  10.1186/1532-429X-13-34

 received in 2011-02-10, accepted in 2011-07-06,  发布年份 2011

来源: Springer

【 摘 要 】

BackgroundThalassaemia major (TM) patients need regular blood transfusions that lead to accumulation of iron and death from heart failure. Deferiprone has been reported to be superior to deferoxamine for the removal of cardiac iron and improvement in left ventricular (LV) function but little is known of their relative effects on the right ventricle (RV), which is being increasingly recognised as an important prognostic factor in cardiomyopathy. Therefore data from a prospective randomised controlled trial (RCT) comparing these chelators was retrospectively analysed to assess the RV responses to these drugs.MethodsIn the RCT, 61 TM patients were randomised to receive either deferiprone or deferoxamine monotherapy, and CMR scans for T2* and cardiac function were obtained. Data were re-analysed for RV volumes and function at baseline, and after 6 and 12 months of treatment.ResultsFrom baseline to 12 months, deferiprone reduced RV end systolic volume (ESV) from 37.7 to 34.2 mL (p = 0.008), whilst RV ejection fraction (EF) increased from 69.6 to 72.2% (p = 0.001). This was associated with a 27% increase in T2* (p < 0.001) and 3.1% increase in LVEF (p < 0.001). By contrast, deferoxamine showed no change in RVESV (38.1 to 39.1 mL, p = 0.38), or RVEF (70.0 to 69.9%, p = 0.93) whereas the T2* increased by 13% (p < 0.001), but with no change in LVEF (0.32%; p = 0.66). Analysis of between drugs treatment effects, showed significant improvements favouring deferiprone with a mean effect on RVESV of -1.82 mL (p = 0.014) and 1.16% for RVEF (p = 0.009). Using regression analysis the improvement in RVEF at 12 months was shown to be greater in patients with lower baseline EF values (p < 0.001), with a significant difference in RVEF of 3.5% favouring deferiprone over deferoxamine (p = 0.012).ConclusionIn this retrospective analysis of a prospective RCT, deferiprone monotherapy was superior to deferoxamine for improvement in RVEF and end-systolic volume. This improvement in the RV volumes and function may contribute to the improved cardiac outcomes seen with deferiprone.

【 授权许可】

Unknown   
© Smith et al; licensee BioMed Central Ltd. 2011. 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.