Right ventricular cardiac dysfunction in beta-thalassemia major
A. Koren, I. Garty, D. Antonelli and E. Katzuni
In patients with iron overload associated with severe,
transfusion-dependent beta-thalassemia, congestive heart failure develops
during the second decade of life. Biventricular heart function was studied
by multigated radionuclide angiography in 22 patients with beta-thalassemia
major. Six patients were symptomatic. Congestive heart failure developed in
five patients at the time of blood transfusions, and one other patient had
been treated for multiple ventricular extrasystole. The mean (+/- SD) left
ventricular ejection fraction was normal (63.0% +/- 7.6%). Only one patient
had a left ventricular ejection fraction under the normal level (less than
50%). The mean (+/- SD) right ventricular fraction (RVEF) was 33.3% +/-
9.4%. In only three patients was the RVEF normal (greater than or equal to
40%); an RVEF under 30% was registered in six patients. We suggest that the
early right ventricular dysfunction in patients with beta-thalassemia may
be due to pulmonary hypertension secondary to iron overload and iron
deposits in the ventricles.
