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European Journal of Heart Failure Advance Access published online on November 4, 2009

European Journal of Heart Failure, doi:10.1093/eurjhf/hfp152
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org.

Cardiac mortality in β-thalassemia major: resting but not dobutamine stress echocardiography predicts mortality among initially cardiac disease-free patients in a prospective 12-year study

George Hahalis1,*, Alexandra Kourakli2, Ioanna Gerasimidou1, Andreas P. Kalogeropoulos1, George Sitafidis1, Urania Papageorgiou3, Periklis Davlouros1, Nikos Grapsas4, Nicholas C. Zoumbos2 and Dimitrios Alexopoulos1

1 Department of Cardiology, Patras University Medical School, 7 Larnakos Street, Rio, Patras 26441, Greece
2 Department of Hematology, Patras University Medical School, Rio, Patras, Greece
3 Department of Hematology, Karamandaneio Hospital, Patras, Greece
4 Department of Cardiology, Agios Andreas Hospital, Patras, Greece

* Corresponding author. Email: ghahalis{at}otenet.gr


   Abstract

Aims: Cardiac death remains the principal cause of mortality in β-thalassemia major (β-TM). Echocardiography may provide additional information, incremental to haematological profile, both for guiding chelation therapy and to assess prognosis.

Methods and results: Between 1993 and 1995, 36 patients with β-TM and normal cardiac function and 25 normal volunteers underwent evaluation using resting and dobutamine stress echocardiography (DSE). Dobutamine stress echocardiography was performed at baseline and repeated after 2 years. The primary endpoint was cardiac mortality. During a 12-year observation period, seven patients (19%) died from heart failure. All seven deaths occurred among the cohort of 12 patients with median ferritin concentrations ≥2800 ng/mg. In addition, a resting left ventricular ejection fraction (LVEF) <60% was also associated with increased late mortality. In multivariate analysis, increased serum ferritin levels and reduced LVEF but not DSE or other haematological variables were independent survival determinants.

Conclusion: Resting LVEF provides prognostic information that is additional to ferritin levels among patients with β-TM.

Key Words: Thalassemia • Heart failure • Echocardiography • Mortality

Received May 10, 2009; Revised August 9, 2009; Accepted August 26, 2009


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