Comparison of cardiac dysfunction in thalassemia major patients using deferoxamine or deferiprone as an iron-chelating agent

  • Rosalina Josep Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Pustika Amalia Wahidiiyat Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Partini Trihono Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Piprim Yanuarso Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Keywords: thalassemia major, deferoxamine, deferiprone, diastolic dysfunction, systolic dysfunction, conventional echocardiography, TDI

Abstract

Background In thalassemia major (TM) patients, major mortality
is due to cardiac hemosiderosis. Several types of iron chelating
agent available recently are given to overcome this problem
Objective To compare cardiac dysfunction in thalassemia major
patients who used subcutaneous deferoxamine (DFO) to those
who used oral deferiprone (DFP) as an ironô€†chelating agent.
Methods This crossô€†sectional study was held at the Thalassemia
Center, Department of Child Healthô€†Cipto Mangunkusumo
Hospital (DCHô€†CMH), Jakarta. We included TM patients aged
1Oô€†18 years Mth a mean preô€†transfused hemoglobin level of 2:.7
g/dL in the prior year, and who had used DFO or DFP for at
least 1 year v.ith good compliance, at a standard dose of DFO at
40-60 mg/kg/day for 5 days a week or DFP at 50-100 mg/kg/day.
We excluded TM patients v.ith congenital heart disease or overt
heart failure. Transô€†thoracal echocardiography was performed at
the Integrated Cardiac Service, CMH by a pediatric cardiologist
using the conventional method and tissue Doppler imaging (TD I)
consecutively, and within 2 weeks of the subject's receiving a
packed red blood cell (PREC) transfusion. The 57 TM subjects
consisted of 19 DFO users and 38 DFP users.
Results In our subjects, diastolic dysfunction was more commonly
seen than systolic dysfunction, especially moderate diastolic
dysfunction. In the DFO group, diastolic dysfunction only was
detected in 3/19 subjects, systolic dysfunction only in 1/19 subjects,
and both diastolic and systolic dysfunction in 15/19 subjects. None
of the DFO users had normal cardiac function. In the DFP group,
diastolic dysfunction only was seen in 6/38 subjects, and both
diastolic and systolic dysfunction in 30/38 subjects, while 2/38
subjects had normal cardiac function.
Conclusion Diastolic and/or systolic dysfunction was detected in
the majority of subjects, but with preserved global cardiac function.
We found that cardiac dysfunction was not significantly different in
the two iron chelator groups. For all subjects, diastolic dysfunction 

was seen in 89% of cases, while systolic dysfunction was detected
in 77% of cases. [Paediatr Indones. 2012;52:272,9].

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Published
2012-10-31
How to Cite
1.
Josep R, Wahidiiyat P, Trihono P, Yanuarso P. Comparison of cardiac dysfunction in thalassemia major patients using deferoxamine or deferiprone as an iron-chelating agent. PI [Internet]. 31Oct.2012 [cited 22Feb.2024];52(5):272-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/473
Received 2016-09-07
Accepted 2016-09-07
Published 2012-10-31