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Cardiac disease in beta-thalassaemia major: Is it reversible?

M Atiq1, M Bana, U S Ahmed

  • 1Department of Paediatrics, The Aga Khan University Hospital, PO Box 3500, Stadium Road, Karachi, Pakistan. mehnaz.atiq@aku.edu

Singapore Medical Journal
|July 26, 2006
PubMed

Insights

Cardiac disease is common in beta-thalassaemia major. Regular chelation therapy can prevent and improve cardiac complications, including systolic and diastolic dysfunction, in these patients.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Beta-thalassaemia major is a genetic blood disorder requiring lifelong transfusions.
  • Iron overload (siderotic disease) is a major complication of transfusions.
  • Cardiac involvement is a significant cause of morbidity and mortality in beta-thalassaemia major.

Purpose of the Study:

  • To assess the range of cardiac problems in patients with beta-thalassaemia major.
  • To determine the outcomes of cardiac involvement in this patient group.

Main Methods:

  • Study included 75 patients (mean age 13.8 years).
  • Evaluated clinical history, physical examination, and laboratory data.
  • Reviewed electrocardiograms, chest radiographs, and echocardiograms.

Main Results:

  • 44 patients (58.7%) had cardiac involvement.
  • Common issues included left ventricular systolic dysfunction (17 patients), diastolic dysfunction (22 patients), pericardial effusion (12 patients), and pulmonary hypertension (12 patients).
  • Intensive chelation therapy and cardiac medications led to improvement in 13/17 patients with systolic dysfunction and 4/22 with diastolic dysfunction.

Conclusions:

  • Cardiac disease is a frequent complication of iron overload in beta-thalassaemia major.
  • Regular chelation therapy is crucial for preventing cardiac complications.
  • This study demonstrated that regular chelation can improve cardiac systolic function.
Abstract

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