© 2007 European Society of Cardiology
Effects of combined deferiprone and desferrioxamine iron chelating therapy in β-thalassemia major end-stage heart failure
a Division of Cardiology Azienda Ospedaliera "G. Brotzu", Via A. Ricchi 1 09134, Cagliari, Italy
b Division of Paediatrics Ospedale "Crobu", ASL 7, Carbonia, Italy
c Department of Imaging Azienda Ospedaliera "G. Brotzu", Cagliari, Italy
d Department of Biomedical Science and Biotechnologies, Ospedale Regionale per le Microcitemie ASL 8, Università degli Studi, Cagliari, Italy
* Corresponding author. Tel.: +39 070 539515; fax: +39 070 531400. E-mail address: maurizioporcu{at}aob.it
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Abstract |
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Despite usual iron chelating therapy based on desferrioxamine, patients affected by β-thalassemia major (β-TM) often develop progressive heart failure caused by myocardial iron overload, which is the leading cause of mortality within the third decade of life. Heart transplantation is a limited therapeutic option, as very often these patients have multi-organ iron deposits and infective complications (particularly hepatitis C), secondary to frequent blood transfusions. We report the case of a 26-year-old male affected by β-TM with end-stage heart failure, who showed a dramatic improvement in symptoms and myocardial function when a new oral iron chelating agent, deferiprone, was added to standard therapy with desferrioxamine.
Key Words: β-Thalassemia • Deferiprone • Heart failure • Heart transplantation
Received June 29, 2006; Accepted August 24, 2006