[show abstract][hide abstract] ABSTRACT: To assess cardiac status in a large group of patients with thalassemia major who had been treated in a standard way since their early infancy with intensive transfusions and deferoxamine chelation therapy and who had good compliance with this regimen.
We assessed clinically and echocardiographically 202 thalassemia major patients aged 27.3 +/- 6.3 yr and 75 age and sex-matched healthy controls. Overt cardiac disease was encountered in 14 patients (6.9%), including 5 (2.5%) with congestive heart failure, aged 26-37 yr, and 9 with systolic left ventricular (LV) dysfunction, aged 23-37 yr. Ten patients (5.0%) had a history of pericarditis. Left atrial and LV diameters, LV mass and cardiac output were significantly higher in patients than in controls, while peripheral resistance and LV afterload were significantly lower. Relative LV wall thickness did not differ between patients and controls, but it was significantly lower in patients with overt cardiac disease compared to those without (P < 0.05). Restrictive LV filling was observed in 37.6% of patients and was significantly more frequent in cases with overt cardiac disease (P < 0.01). Pulmonary hypertension was practically absent. Hematological parameters and pulmonary artery pressure levels were not independently associated with the presence of overt cardiac disease.
Strict lifelong adherence to the standard transfusion and deferoxamine therapy reduces considerably the occurrence of heart failure, LV dysfunction and pericarditis, prevents early heart failure and pulmonary hypertension, but does not eliminate completely cardiac disease in patients with thalassemia major.
European Journal Of Haematology 12/2004; 73(5):359-66. · 2.55 Impact Factor
[show abstract][hide abstract] ABSTRACT: 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 not been transfused or were minimally transfused (group A). The rest had started transfusions after the age of 5 years (mean, 15.1 +/- 10.1 years), initially on demand and later more frequently (group B). Overall mean hemoglobin and ferritin levels were 9.1 +/- 1.1 g/dL and 1657 +/- 1477 ng/mL, respectively. 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 acute pericarditis. Echocardiography showed pericardial thickening, with or without effusion, in 34.5% of the patients. Valvular involvement included leaflet 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 Hg, developed in 65 patients (59.1%). PHT correlated positively with age and CO and did not differ significantly between groups. Cardiac catheterization in the 6 patients with CHF revealed severe PHT, increased pulmonary resistance (PVR), and normal capillary wedge pressure. It was concluded that in patients with TI, the heart is primarily affected by PHT, which is the leading cause of CHF. High CO resulting from chronic tissue hypoxia and increased PVR are the main contributing factors. Doppler tricuspid gradient measurement should be considered, in addition to other factors, when determining the value of transfusion therapy for patients with TI. (Blood. 2001;97:3411-3416)