Cardiac disease in beta-thalassaemia major: Is it reversible?
BACKGROUND
The aim of this study was to evaluate the spectrum of cardiac involvement and its outcome in beta-thalassaemia major.


METHODS
There were 75 patients with a mean age of 13.8 (+/- 5.5) years, of whom 33 were male and 42 were female. Clinical history, examination and laboratory investigations were assessed. Electrocardiograms, chest radiographs and echocardiograms were reviewed.


RESULTS
44 patients had cardiac involvement in the form of left ventricular systolic dysfunction in 17, diastolic dysfunction in 22, pericardial effusion in 12 and pulmonary hypertension in 12 patients. With intense chelation therapy and cardiac medications, the condition of 13 of 17 patients with systolic dysfunction, and four of 22 with diastolic dysfunction, improved.


CONCLUSIONS
Cardiac disease is a common complication of siderotic disease in thalassaemia major and it can be prevented with regular chelation. This study has shown improved systolic function after regular chelation therapy.
Atiq
M
M
Department of Paediatrics, The Aga Khan University Hospital, PO Box 3500, Stadium Road, Karachi, Pakistan. mehnaz.atiq@aku.edu
Bana
M
M
Ahmed
U S
US
Bano
S
S
Yousuf
M
M
Fadoo
Z
Z
Khurshid
M
M
D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002316:Cardiotonic Agents / Q000627:therapeutic use; D002318:Cardiovascular Diseases / Q000188:drug therapy / Q000209:etiology*; D015913:Chelation Therapy; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood / Q000172:deficiency; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D017262:Siderophores / Q000627:therapeutic use; D013599:Systole; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy
D002316:Cardiotonic Agents; D017262:Siderophores; D005293:Ferritins; D003676:Deferoxamine