Email updates

Keep up to date with the latest news and content from Journal of Cardiovascular MR and BioMed Central.

Open Access Highly Accessed Research

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

Gillian C Smith12, Francisco Alpendurada1, John Paul Carpenter12, Mohammed H Alam12, Vasili Berdoukas3, Markissia Karagiorga4, Vasili Ladis4, Antonio Piga5, Athanassios Aessopos6, Efstathios D Gotsis7, Mark A Tanner8, Mark A Westwood9, Renzo Galanello10, Michael Roughton11 and Dudley J Pennell12*

Author Affiliations

1 CMR Unit, Royal Brompton & Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK

2 National Heart & Lung Institute, Imperial College London, Guy Scadding Building, Dovehouse Street, London SW3 6LY, UK

3 Division of Haematology/Oncology, Children's Hospital, 4650 Sunset Boulevard, Los Angeles, CA 90027, USA

4 Thalassaemia Unit, Aghia Sophia Children's Hospital, Thivon & Papadiamantopoulou, Goudi, Athens 115 27, Greece

5 Division of Paediatrics and thalassaemia centre, Department of Clinical and Biological Sciences, University of Torino, S. Luigi Gonzaga Hospital, Regione Gonzole 10, Orbassano 10043, Torino, Italy

6 First Department of Internal Medicine, University of Athens Medical School, Laiko Hospital, 17 Agiou Thoma Street, Athens 115 27, Greece

7 Department of Magnetic Resonance, Institute Euromedica-Encephalos, 3 Rizariou Street, Halandri, Athens 152 33, Greece

8 Department of Cardiology, St Richard's Hospital, Western Sussex Hospitals NHS Trust, Chichester, West Sussex, PO19 6SE UK

9 Department of Cardiology, The London Chest Hospital, Bonner Road, London E2 9JX, UK

10 Department of Biomedical Science and Biotechnology, University of Cagliari, Ospedale Regionale per le Microcitemie. Via Edward Jenner, 09121 Cagliari, Italy

11 UCL Cancer Trials Centre, University College London, 90 Tottenham Court Road, London W1T 4TJ, UK

For all author emails, please log on.

Journal of Cardiovascular Magnetic Resonance 2011, 13:34  doi:10.1186/1532-429X-13-34

Published: 6 July 2011

Abstract

Background

Thalassaemia 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.

Methods

In 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.

Results

From 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).

Conclusion

In 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.