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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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
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 |
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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