Cardiovascular effects of splenomegaly and splenectomy in β-thalassemia

Abstract

Splenomegaly is common in beta-thalassemia, bearing some particular hemodynamic features, while splenectomy is an established therapeutic intervention in these patients. Their effects, however, on systemic hemodynamics and thalassemia heart disease have not yet been assessed. The study included 32 consecutive patients, 13 with thalassemia major (TM) and 19 with thalassemia intermedia (TI), aged 23.4+/-4.2 years, requiring splenectomy. Assessment was performed before and 6 months after splenectomy and included hematological profile and resting echocardiography; total blood volume was also measured in 10 of 32 cases. Preoperative echocardiographic data were compared with those of 34 controls. Preoperative left ventricular diameters and mass, cardiac index, and systolic pulmonary artery pressure were all significantly higher in patients compared to controls (p<0.001), but did not differ significantly between TM and TI patients. Postoperatively, the mean hemoglobin level increased from 8.1+/-0.6 to 9.1+/-0.4 g/dl (p<0.001), total blood volume index declined from 2847+/-332 to 2310+/-276 ml/m(2) (p<0.001), blood transfusions were discontinued in 80% of TI patients and mean 6-monthly transfusion requirements in TM patients were reduced from 28+/-5 to 22+/-4 units (p<0.001). However, cardiac parameters were not significantly modified. It seems that left ventricular remodeling, high output state, and increased pulmonary artery pressure characterize both TM and TI patients who require splenectomy. Although these abnormalities remain unchanged after splenectomy, the removal of the spleen may contribute to the prevention of further cardiac damage by ameliorating the patients' hematological status and reducing their transfusion needs.