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High incidence of cardiomyopathy in beta-thalassaemia patients receiving regular transfusion and iron chelation: reversal by intensified chelation
- Source :
- Acta haematologica. 84(3)
- Publication Year :
- 1990
-
Abstract
- Cardiac scintigraphy has been performed in 60 beta-thalassaemia major patients aged 8-35 years who received regular blood transfusions and subcutaneous desferrioxamine (DFX) chelation. Fifty-seven showed no clinical, radiological or electrocardiographic evidence of heart disease and 3 had clinically apparent cardiac failure. Twenty-two patients (37%) showed severe cardiac functional impairment defined by a resting left ventricular ejection fraction (LVEF) less than 45% and/or a drop of greater than 12% on stress, while 19 were normal and 19 had a mild abnormality. There was no significant correlation between abnormality of LVEF and age, serum ferritin, number of units transfused, dose and duration of subcutaneous DFX therapy, liver disease or sexual maturation. Non-compliant patients (defined as the use of subcutaneous DFX less than 4 times weekly) generally showed worse cardiac function. Repeat study on 17 patients after 6-28 months of better compliance with subcutaneous or intravenous DFX (using an indwelling catheter) showed a significant overall improvement in LVEF associated with a significant drop in serum ferritin. We conclude that cardiac scintigraphy uncovers a high incidence of cardiac functional abnormality in asymptomatic, well-transfused thalassaemia patients, particularly those poorly compliant with chelation. Those with poor LVEF results should be offered intensive chelation therapy to improve cardiac function.
- Subjects :
- Adult
Male
medicine.medical_specialty
Adolescent
Heart Diseases
medicine.medical_treatment
Cardiomyopathy
Deferoxamine
Gastroenterology
Iron chelation
Internal medicine
medicine
Humans
Chelation
Blood Transfusion
Child
Radionuclide Imaging
Chemotherapy
business.industry
Incidence
Stroke Volume
Hematology
General Medicine
medicine.disease
Combined Modality Therapy
Chelation Therapy
Surgery
Hemoglobinopathy
Thalassemia
Female
High incidence
Complication
business
medicine.drug
Follow-Up Studies
Subjects
Details
- ISSN :
- 00015792
- Volume :
- 84
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Acta haematologica
- Accession number :
- edsair.doi.dedup.....ec4a59c3cf52b1821e45db3555a22787