Survival in Thalassaemia Major Patients : Prognostic Value of Doppler-Demonstrated Left Ventricular Restrictive Filling Pattern

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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. Methods and 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=0.01 8). 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).
社団法人日本循環器学会の論文
2006-07-20