PULMONARY-HYPERTENSION IN PATIENTS WITH THALASSEMIA MAJOR

To evaluate the pulmonary artery pressure in patients with thalassemia major, Doppler echocardiography was performed in 33 patients with tha lassemia major (aged 2 to 24 years) and 33 normal controls. Pulmonary artery pressure was estimated by ii) measuring the systolic transtricu spid gradient From tricuspid regurgitation and adding it to the right atrial pressure, estimated by the response of the inferior vena cava t o deep inspiration, and (2) measuring the time to peak velocity of pul monary flow, The results showed that oi 33 patients, 28 had tricuspid regurgitation with a pulmonary systolic pressure ranging from 18 to 94 mm Hg (47 +/- 15 mm Hg). Pulmonary systolic pressure was >30 mm Hg in all 22 patients >10 years old and in four of six patients <10 years o ld. Pressure correlated with left ventricular ejection fraction (r=-0. 74), the ratio of mitral peak early diastolic flow velocity and peak v elocity during the atrial contraction (r=0.52), age (r=0.56), and tota l blood transfusion units (r=0.59). The pulmonary time to peak velocit y was shortened compared with controls (p < 0.05), We concluded that p ulmonary hypertension, as another cardiovascular complication of multi ple factors of cause, seems to occur more frequently and at an early s tage of the cardiac involvement in patients with thalassemia major.