Survival in thalassaemia major patients.
BACKGROUND
The present study evaluated the prognostic significance of Doppler-demonstrated left ventricular (LV) restrictive filling pattern (RFP) in patients with thalassaemia major (TM), which carries an adverse cardiovascular prognosis.


RESULTS
The study group comprised 45 asymptomatic transfusion-dependent patients with TM and normal LV systolic function. All patients were chelated with desferrioxamine. They were regularly evaluated by clinical and Doppler-echocardiographic studies throughout the 15-year follow-up period. The patients were categorized into 2 groups according to baseline data: those with LVRFP and those with LV non-RFP. The incidence of cardiac death in both groups was analyzed. The impact of chelation therapy on the ventricular filling pattern and survival was also examined. Nineteen patients (42.2%) had LVRFP and 26 (57.8%) had LV non-RFP. During follow-up 11 patients died from cardiac causes; 8 of them (72.8%) initially had LVRFP and 3 (27.2%) had LV non-RFP. LVRFP was significantly associated with mortality (p=0.018). Poor compliance with chelation therapy was significantly associated with LVRFP (p=0.007) and cardiac mortality (p=0.003).


CONCLUSIONS
LVRFP is an important predictor of cardiac mortality in patients with TM. Poor compliance with chelation therapy was significantly associated with both a RFP (p=0.007) and cardiac mortality (p=0.003).
Efthimiadis
Georgios K
GK
Department of Cardiology, Aristotle University, and Thalassemia Unit, AHEPA Hospital, Thessaloniki, Greece. efthymos@med.auth.gr
Hassapopoulou
Helen P
HP
Tsikaderis
Dimitrios D
DD
Karvounis
Haralambos I
HI
Giannakoulas
Georgios A
GA
Parharidis
Georgios E
GE
Louridas
Georgios E
GE
D000293:Adolescent; D000328:Adult; D015913:Chelation Therapy; D003643:Death; D003676:Deferoxamine / Q000627:therapeutic use; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D017262:Siderophores / Q000627:therapeutic use; D016019:Survival Analysis; D015996:Survival Rate; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology*; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000401:mortality*
D017262:Siderophores; D003676:Deferoxamine