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

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.