Hahalis G, Kourakli A, Gerasimidou I, Kalogeropoulos AP, Sitafidis G, Papageorgiou U, Davlouros P, Grapsas N, Zoumbos NC, Alexopoulos D
Cardiac mortality in {beta}-thalassemia major: resting but not dobutamine stress echocardiography predicts mortality among initially cardiac disease-free patients in a prospective 12-year study. [JOURNAL ARTICLE]
Eur J Heart Fail 2009 Nov 4.
AIMS: Cardiac death remains the principal cause of mortality in beta-thalassemia major (beta-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 beta-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 beta-TM.
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