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Cardiac dysfunction in beta-thalassemic children

P Chotivittayatarakorn1, P Seksarn, C Pathmanand

  • 1Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Journal of the Medical Association of Thailand = Chotmaihet Thangphaet
|November 1, 1993
PubMed
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In children with beta-thalassemia, right ventricular dysfunction appears before left ventricular issues. Platelets may contribute to pulmonary artery hypertension development in these patients.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Cardiovascular Research

Background:

  • Beta-thalassemia and beta-thalassemia Hb E disease are inherited blood disorders.
  • Cardiac complications, including ventricular dysfunction and pulmonary hypertension, can occur in these patients.

Purpose of the Study:

  • To evaluate biventricular systolic function in children with beta-thalassemia.
  • To investigate the relationship between platelet count and pulmonary artery hypertension.

Main Methods:

  • M mode and Doppler echocardiography were used to assess cardiac function.
  • 16 patients aged 5-14 years with homozygous beta-thalassemia or beta-thalassemia Hb E disease were studied.

Main Results:

  • Increased left ventricular end-diastolic dimension and mass were observed in 88% of patients.

Related Experiment Videos

  • Pulmonary artery hypertension was evident in 15 patients, indicated by abnormal right ventricular acceleration time to ejection time (AT/ET) ratio.
  • A significant correlation was found between platelet count and AT/ET (r = -0.70, P = 0.002), with thrombocytosis in 4 patients.

    • Conclusions:

    • Right ventricular dysfunction is detected earlier than left ventricular dysfunction in children with beta-thalassemia.
    • Platelets may play a role in the pathogenesis of pulmonary artery hypertension in this population.