Τίτλος:
Cardiac status in well-treated patients with thalassemia major
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Objective: 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. Methods and
Results: 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. Conclusion:
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.
Συγγραφείς:
Aessopos, A
Farmakis, D
Hatziliami, A
Fragodimitri, C and
Karabatsos, F
Joussef, J
Mitilineou, E
Diamanti-Kandaraki, E
and Meletis, J
Karagiorga, M
Περιοδικό:
European Journal of Haematology
Λέξεις-κλειδιά:
thalassemia major; heart disease; heart failure; iron overload; iron
chelation; deferoxamine
DOI:
10.1111/j.1600-0609.2004.00304.x
