Cardiac involvement in thalassemia intermedia: a multicenter study

Cardiac complications in 110 patients (mean age, 32.5 +/- 11.4 years) with thalassemia intermedia (TI) were studied. Sixty-seven (60.9%) of them had n ot been transfused or were minimally transfused (group A). The rest had sta rted transfusions after the age of 5 years (mean, 15.1 +/- 10.1 years), ini tially on demand and later more frequently (group B), Overall mean hemoglob in and ferritin levels were 9.1 +/- 1.1 g/dL and 1657 +/- 1477 ng/mL, respe ctively. Seventy-six healthy controls were also studied, The investigation included thorough history taking, clinical examination, electrocardiography , chest radiograph, and full resting echocardiography. Of 110 patients, 6 ( 5.4%) had congestive heart failure (CHF), and 9 (8.1%) had a history of acu te pericarditis. Echocardiography showed pericardial thickening, with or wi thout effusion, in 34.5% of the patients. Valvular involvement included lea flet thickening (48.1%), endocardial calcification (20.9%), and left-sided valve regurgitation (aortic, 15.4%; mitral, 47.2%), All patients had normal left ventricular contractility (fractional shortening, 0.43 +/- 0.05), and high cardiac output (CO; 9.34 +/- 2.28 L/min). Pulmonary hypertension (PHT ), defined as Doppler peak systolic tricuspid gradient greater than 30 mm H g, developed in 65 patients (59.1%), PHT correlated positively with age and CO and did not differ significantly between groups. Cardiac catheterizatio n in the 6 patients with CHF revealed severe PHT, increased pulmonary resis tance (PVR), and normal capillary wedge pressure. It was concluded that in patients with TI, the heart is primarily affected by PHT, which is the lead ing cause of CHF, High CO resulting from chronic tissue hypoxia and increas ed PVR are the main contributing factors. Doppler tricuspid gradient measur ement should be considered, in addition to other factors, when determining the value of transfusion therapy for patients with TI, (Blood. 2001; 97:341 1-3416) (C) 2001 by The American Society of Hematology.