American Heart Association Releases Statement on Cardiac Dysfunction in Thalassemia Major

June 20, 2013 – The American Heart Association (AHA)  has released a consensus statement on “Cardiovascular Function and Treatment in Beta-Thalassemia Major.”

Published online in the AHA journal Circulation, the document states in part that “central to early identification of cardiac iron overload in TM (thalassemia major) is the estimation of cardiac iron by cardiac T2* magnetic resonance.  Cardiac T2*<10 ms is the most important predictor of development of heart failure.”

The article also states that “assessment of cardiac function by noninvasive techniques can also be valuable clinically, but serial measurements to establish trends are usually required.”

The AHA statement also points out that acute heart failure in thalassemia major requires expert management; the first  principle of treatment in such cases should be commencement of “continuous uninterrupted infusion of high-dose intravenous deferoxamine, augmented by oral deferiprone.”

In cases in which there is cardiac iron overload without overt cardiac dysfunction, the paper refers to studies which show “superior efficacy of deferiprone versus deferoxamine, the superiority of combined deferiprone with deferoxamine versus deferoxamine alone, and the equivalence of deferasirox versus deferoxamine.”

CAF has long been a supporter of the value of noninvasive MRI-based techniques for assessing cardiac iron and its impact on heart function.  If the center where you receive treatment does not have the specialized technology required for conducting T2* measurements, please contact CAF Patient Services Manager Eileen Scott at (212) 279-8090 or eileen.s@thalassemia.org.

The Foundation thanks the authors of this consensus statement for this important contribution to the fund of knowledge concerning thalassemia and its treatments.  Those authors are Dudley J. Pennell, MD, FRCP, FAHA; James E. Udelson, MD, FAHA; Andrew E. Arai, MD, FAHA; Biykem Bozkurt, Md, PhD, FAHA; Alan R. Cohen, MD; Renzo Galanello, MD; Timothy M. Hoffman, MD, FAHA; Michael S. Kiernan, MD; Stamatios Lerakis, MD, FAHA; Antonio Piga, MD; John B. Porter, MD; John Macolm Walker, MD; and John Wood, MD, PhD.

The AHA concensus statement may be read in its entirety or downloaded by clicking here.

 

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