CARDIAC INVOLVEMENT IN MAJOR-THALASSEMIA

Heart failure is the most common cause of death in patients (pts) with Thalassemia Major (TM), usually in the 3rd-4th decade of life. 26 pts with TM were studied to assess: 1) the usefullness of Doppler Echocar diography (DE), 24 hour ECG-monitoring (ECG-M) and Late Potentials on high resolution ECG for detection of early cardiac involvement; 2) the efficacy of chelation therapy with deferoxamine (D) in preventing, ca rdiac disease. RESULTS: a) left ventricular and left atrial diameter/m 2 are significantly increased in pts with TM (also in asymptomatic pts ) vs normal controls; b) diastolic indexes (from transmitral flow) are not early compromised; c) ventricular arrhythmias, possibly life-thre atening- can be detected with ECG-M; d) a depressed systolic function, associated with symptoms of heart failure, appears more frequently in pts with poor compliance to chelation therapy. Our experience underli es both the usefulness of DE and ECG-M in cardiac follow-up of pts wit h TM, and the importance of a scrupulous chelation therapy for the pre vention of early cardiac disease.