Dudley Pennell
MD FRCP FACC FESC

How can MRI be used to optimize the management of thalassaemia?

Learning Objectives

  • Learn how MRI T2* can be used to monitor cardiac function and prevent cardiac mortality from iron loading of the heart.
  • Gain insight into how MRI T2* can assist clinicians in optimizing their patients treatment
  • Review the effect of different iron chelators at removing iron from the heart
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Cardiac disease is the cause of approximately 70 percent of deaths in beta thalassaemia major. Cardiovascular magnetic resonance (CMR) can measure cardiac iron deposition through the magnetic relaxation parameter T2*. This allows the determination of best chelation strategies for the heart by direct assessment of the clearance of myocardial iron.

Deferoxamine (constant intravenous) was assessed in seven patients presenting with heart failure. Of the six survivors, cardiac T2* improved significantly over one year, but clearance was very slow. A case-controlled study comparing deferiprone with deferoxamine showed lower myocardial iron and superior ejection fraction in patients on deferiprone. Two prospective, randomized, controlled, blinded trials have been conducted to explore this result further. The first trial compared deferiprone 100mg with deferoxamine and showed superior efficacy for deferiprone in removing cardiac iron. The second trial compared combination therapy vs. deferoxamine alone, and is now being analyzed.

Direct measures of myocardial iron using myocardial T2* are giving new insights into cardiac deaths in thalassaemia. We are working with TIF and NIH grants to validate other centres worldwide and to calibrate the method. So far, oral chelation with deferiprone appears to be more effective than deferoxamine in removing cardiac iron.