Echocardiographic finding in beta-thalassemia intermedia and major: absence of pulmonary hypertension following hydroxyurea treatment in beta-thalassemia intermedia.

OBJECTIVES: The aim of this study was to investigate the echocardigraphic finding in beta-thalassemia intermedia (TI) and beta-thalassemia major (TM) and to compare this finding together and with healthy control subjects.

METHODS: Fifty TI, who have been treated with hydroxyurea (HU) for 7 yrs and 51 transfusion dependent TM were compared with 50 age and sex matched healthy control subjects. Left and right ventricular parameters, systolic and diastolic functions, stroke volume, cardiac index and indices of pulmonary hypertension (PHT) were determined by two-dimensional, M-mode echocardiography and Doppler echocardiography.

RESULTS: Left ventricular parameters such as left ventricular end diastolic diameter, left ventricular end systolic diameter and also interventricular septal diameter in systole and diastole were significantly higher in TI patients compared with TM and control group (P < 0.05). There was elevated left ventricular mass (LV mass) in TI and TM patients compared with controls (P < 0.05). Regarding the LV diastolic function indices, E and A were significantly higher in TI patients compared with TM patients and control which were compatible with high output state. Measurement of pulmonary acceleration time and tricuspid and pulmonary valve continuous-wave Doppler tracing in patients with tricuspid regurgitation and pulmonary insufficiency showed no difference between TI, TM and control group.

CONCLUSION: Both TI and TM patients who have no clinical signs of cardiac involvement have significant abnormalities in volume, mass and shape of the LV which may be the consequence of chronic anemia. We found the unexpected absence of PHT in TI patients who have been treated with HU. In conclusion Low dose HU treatment of TI patients may prevent the devastating complication of PHT.