Cardiac Involvement in β‐Thalassemia Major and β‐Thalassemia Intermedia

Abstract

The forms and severity of cardiac complications were investigated in patients with asymptomatic thalassemia intermedia and thalassemia major by M‐mode, bi‐dimensional echocardiography (ECHO) and echo‐Doppler. Twenty‐eight patients of both sexes with β‐thalassemia intermedia (β‐TI), mean age 23.2 ± 6.3 years, untransfused or minimally transfused, were compared to 42 age‐ and sex‐matched subjects with thalassemia major, who were regularly treated with hemotransfusive therapy [pre‐transfusion hemoglobin (Hb) values 9.5 ± 0.9 g/dL] and iron chelation. All patients were splenectomized. Age and sex matched healthy control subjects were randomly selected. β‐Thalassemia major (β‐TM) patients showed a marked reduction in contractile state and a milder left ventricular (LV) enlargement than β‐TI patients. Cardiac output (CO) and cardiac index (CI) were increased in both groups of patients but appeared significantly higher in β‐TI patients with consequent altered LV diastolic function indices. In addition, β‐TI patients had reduced indices of pulmonary artery flow related to long‐term chronic anemia rather than iron overload. The progressive rise in CO and CI casts doubts on the current management of β‐TI syndromes.

• β‐Thalassemia intermedia (β‐TI)
• β‐Thalassemia major (β‐TM)
• Cardiac output (CO)
• Cardiac index (CI)