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 thalassemia major (aged 2 to 24 years) and 33 normal controls. Pulmonary artery pressure was estimated by (1) measuring the systolic transtricuspid gradient from tricuspid regurgitation and adding it to the right atrial pressure, estimated by the response of the inferior vena cava to deep inspiration, and (2) measuring the time to peak velocity of pulmonary flow. The results showed that of 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 old. Pressure correlated with left ventricular ejection fraction ( r = -0.74), the ratio of mitral peak early diastolic flow velocity and peak velocity during the atrial contraction ( r = 0.52), age ( r = 0.56), and total blood transfusion units ( r = 0.59). The pulmonary time to peak velocity was shortened compared with controls ( p < 0.05). We concluded that pulmonary hypertension, as another cardiovascular complication of multiple factors of cause, seems to occur more frequently and at an early stage of the cardiac involvement in patients with thalassemia major. (Am Heart J 1997;134:532-7.)