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.
OBJECTIVE
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.


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 >or= 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.


CONCLUSIONS
Resting LVEF provides prognostic information that is additional to ferritin levels among patients with beta-TM.
Hahalis
George
G
Department of Cardiology, Patras University Medical School, Rio, Patras, Greece. ghahalis@otenet.gr
Kourakli
Alexandra
A
Gerasimidou
Ioanna
I
Kalogeropoulos
Andreas P
AP
Sitafidis
George
G
Papageorgiou
Urania
U
Davlouros
Periklis
P
Grapsas
Nikos
N
Zoumbos
Nicholas C
NC
Alexopoulos
Dimitrios
D
D000328:Adult; D025401:Echocardiography, Stress; D005260:Female; D005293:Ferritins / Q000097:blood; D006331:Heart Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000150:complications / Q000000981:diagnostic imaging; D008137:Longitudinal Studies; D008297:Male; D011379:Prognosis; D016277:Ventricular Function, Left; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications*
D005293:Ferritins