January 1987

Right Ventricular Cardiac Dysfunction in β-Thalassemia Major

Author Affiliations

From the Departments of Pediatrics B (Drs Koren [Kurlat] and Katzuni), Cardiology (Dr Antonelli), and Nuclear Medicine (Dr Garty), Central Emek Hospital, Afula, Israel.

Am J Dis Child. 1987;141(1):93-96. doi:10.1001/archpedi.1987.04460010093035

• In patients with iron overload associated with severe, transfusion-dependent β-thalassemia, congestive heart failure develops during the second decade of life. Biventricular heart function Was studied by multigated radionuclide angiography In 22 patients with β-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 (<50%). The mean (± SD) right ventricular fraction (RVEF) was 33.3%±9.4%. In only three patients Was the RVEF normal (≥40%); an RVEF under 30% was registered in six patients. We suggest that the early right ventricular dysfunction in patients with β-thalassemia may be due to pulmonary hypertension secondary to iron overload and iron deposits in the ventricles.

(AJDC 1987;141:93-96)