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.