The correlation between ferritin level and cardiac dysfunction in patients with thalassemia

  • Fajar Subroto
  • Bulan Ginting Munthe
  • Najib Advani
  • Agus Firmansyah
Keywords: thalassemia, iron overload, ferritin, cardiac dysfunctions, ECG, echocardiography

Abstract

Background Patients with b-thalassemia major, long-term trans-
fusion, extravasal hemolytic, and increased intestinal absorption
of iron result in systemic iron overload, which may accumulate in
myocardium causing cardiac dysfunctions. Congestive heart fail-
ure usually develops in adolescence or early adult years, and pa-
tient usually dies within 1 year after the onset of symptoms. There-
fore, it is important to detect early signs of cardiac dysfunction in
patient with thalassemia.
Objective This study aimed to assess the correlation between
ferritin level and cardiac dysfunction in patients with thalassemia.
Methods A cross sectional study was conducted on 62 b-thalas-
semic patients (34 males and 28 females) with age ranging from
3.5 to 23 years. They received 3,150 – 94,985 mL of blood; with
the serum ferritin level of 812.2 – 12,175 ng/mL. Each patient un-
derwent laboratory examinations and clinical cardiac evaluation
with ECG and echocardiography.
Results Cumulative blood transfusions correlated well with the
serum ferritin values (p=0.001). The serum ferritin level did not
show correlation with deferoxamine (DFO) treatment. ECG exami-
nation revealed 5 patients (8.1%) with dysrhythmia, LVH, and heart
block grade II and I. Echocardiography examination showed 18
patients (29%) with systolic or diastolic dysfunction. There was no
correlation between the serum ferritin level and cardiac dysfunc-
tions (p=0.640). The serum ferritin prediction value against car-
diac dysfunctions could not be established.
Conclusion There was no correlation between serum ferritin lev-
els with cardiac dysfunctions. In detecting cardiac dysfunctions in
thalassemic patients, echocardiography was more sensitive than
ECG

Author Biographies

Fajar Subroto
Division of Hematology and Cardiology, Department of Child
Health, Medical School, University of Indonesia—Cipto Mangunkusumo Hospital, Jakarta.
Bulan Ginting Munthe
Division of Hematology and Cardiology, Department of Child
Health, Medical School, University of Indonesia—Cipto Mangunkusumo Hospital, Jakarta.
Najib Advani
Division of Hematology and Cardiology, Department of Child
Health, Medical School, University of Indonesia—Cipto Mangunkusumo Hospital, Jakarta.
Agus Firmansyah
Division of Hematology and Cardiology, Department of Child
Health, Medical School, University of Indonesia—Cipto Mangunkusumo Hospital, Jakarta.

References

1. Spirito P, Lupi G, Melevendi C, Vecchio C. Restrictive
diastolic abnormalities identified by Doppler echo-
cardiography in patients with thalassemia major. Cir-
culation 1990; 82:88-94.
2. Weatherall DJ, Clegg JB. The β thalassemias. In:
Weatherall DJ, Clegg JB, editors. The thalassaemia
syndromes. 3 rd ed. London: Blackwell scientific publi-
cations; 1981. p. 148-319.
3. Powell LW, George DK, McDonnell SM, Kowdley KV.
Diagnosis of hemochromatosis. Ann Intern Med
1998;129:925-31.
4. Barton JC, McDonnell SM, Adam PC, Brissot P, Powell
LW, Edwards CQ, et al. Management of hemochroma-
tosis. Ann Intern Med 1998; 129:932-9.
5. McDonagh KT, Nenhuis AW. The thalassemias. In:
Nathan DG, Oski FA, editors. Hematology of infancy
and childhood. 4 th ed. Philadelphia: WB Saunders;
1993. p. 783-857.
6. Worwood M. Pathogenesis and management of
haemochromatosis. Br J Haematol 1999;105: 16-8.
7. Lau KC, Li AMC, Hui PW, Yeung CY. Left ventricular
function in β thalasaemia major. Arch Dis Child 1989;
64:1046-51.
8. Edwards CQ, Kushner JP. Screening for hemochroma-
tosis. N Engl J Med 1993; 328:1616-20.
9. Pippard NE. Iron loading and chelation therapy. In:
Weatherall DJ, editor. The thalassemias. New York:
Churchill Livingstone; 1983. p. 103-13.
10. Garcia P. Iron in today’s laboratory. Clin Biochemist
Rev 1997; 18:113-7.
11. Ehlers KH, Giardina PJ, Lesser ML, Engle MA,
Hilgartner MW. Prolonged survival in patients with
beta-thalassemia major treated with deferoxamine. J
Pediatr 1991; 118:540-5.
12. Kremastinos DT, Tsiapras DP, Tsetsos GA, Rentoukas
EI, Vretou HP, Toutouzas PK. Left ventricular dias-
tolic Doppler characteristics in b-thalassemia major.
Circulation 1993; 88:1127-35.
13. Leon MB, Borer JS, Bacharach SL, Green MV, Benz
EJ, Griffith P, et al. Detection of early cardiac dysfunc-
tion in patients with severe beta-thalassemia and
chronic iron overload. N Engl J Med 1979; 22:1143-8.
14. Olivieri NF, Brittenham GM. Iron-chelating therapy and
the treatment of thalasemia. Blood 1997; 89:739-54.
15. Brittenham GM, Griffith PM, Nienhuis AW,
McLaren CE, Young NS, Tucher EE, et al. Efficacy
of deferoxamine in preventing complications of iron
overload in patients with thalassemia major. N Eng
J Med 1994; 331:567-73.
16. Olivieri NF, Nathan DG, Mac Millan JH, Wayne AS,
Liu AP, McGee A, et al. Survival in medically treated
patients with homozygous b-thalassemia. N Engl J Med
1994; 331:574-8.
17. Kremastinos DT, Tiniakos G, Theodorakis GN, Katritsis
DG, Toutouzas PK. Circulation 1995; 91;66-71.
18. Koren A, Garty itzhak, Antonelli D, Katzumi E. Right
ventricular cardiac dysfunction . Am J Dis Child 1987;
141:93-6.
Published
2016-09-24
How to Cite
1.
Subroto F, Munthe B, Advani N, Firmansyah A. The correlation between ferritin level and cardiac dysfunction in patients with thalassemia. PI [Internet]. 24Sep.2016 [cited 12Aug.2022];43(1):24-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/654
Section
Articles
Received 2016-09-22
Accepted 2016-09-22
Published 2016-09-24