The occurrence of pulmonary hypertension in patients with thalassemia major
Keywords:
pulmonary hypertension, thalassemia major, blood transfusion, echocardiography
Abstract
Background The life of patients with thalassemia major dependson blood transfusions, while repeated blood transfusions may cause
adverse effects such as iron deposition in various organs, includ-
ing heart and lungs, which eventually increases the pulmonary
arterial pressure.
Objective This study was proposed to know the occurrence of
pulmonary hypertension in patients with thalassemia major, mea-
sured by echocardiography in the Thalassemia Clinic, Department
of Child Health, Medical School, Padjadjaran University/Hasan
Sadikin Hospital, Bandung.
Methods A descriptive cross-sectional study was carried out
on 30 patients with thalassemia major, aged 10-14 year-old who
received repeated blood transfusions. The study was conducted
from April to May 2002. Subjects were examined right after a
blood transfusion completed and the pulmonary arterial pres-
sure was assessed using Doppler–echocardiography and 2-D
echocardiography.
Results Twenty two out of 30 subjects showed pulmonary hyper-
tension, with pulmonary arterial pressure ranged between 32.3 to
46.2 mmHg. According to the age group, pulmonary hypertension
was found in 12 out of 17 subjects aged 10-12 years old and 10
out of 13 subjects aged 13-14 years old.
Conclusion The occurrence of pulmonary hypertension in patients
with thalassemia major at Hasan Sadikin Hospital was 22/30 and
seemed to increase with the age of the patients
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major. Am Heart J 1997;134:532-7.
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Hilgartner MW. Prolonged survival in patients with
beta-thalassemia major treated with deferoxamine. J
Pediatr 1991;118:540-5.
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& Wilkins; 1994. p. 93-118
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diology for practitioners. 3 rd ed. St Louis: Mosby;
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Pediatric in review 1992;13:55-63.
2. Benz EJ, Giardina PJV. Thalassemia syndrome. In:
Miller DR, editor. Blood diseases of infancy and child-
hood. Mosby Year book Inc. St Louis; 1995. p. 460-98.
3. Honig GR. Hemoglobin Disorders. In: Behrman,
Kleigman, Arvin, editors. Nelson textbook of pediat-
rics. 15 th ed. Philadelphia WB Saunders Co; 1996. p.
1401-5
4. Olivieri NF. The beta thalassemias. N Engl J Med
1999;341:99-109.
5. Olivieri NF, Nathan DG, MacMillan JH, Wayne AS,
Liu PP, Mc Gee A, et al. Survival in medically treated
patients with homozygous beta – thalassemia. N Eng
J Med 1994;331:574-8.
6. Leon MB, Borer JS, Bacharach SL, Green MV, Benz EJ
Jr, Griffith P, et al. Detection of early cardiac dysfunc-
tion in patients with severe beta-thalassemia and
chronic iron overload. N Eng J Med 1979;301:1143-8.
7. Zurlo MG, De Stefano S, Borgna-Pignatti C, Di Palma
A, Piga A, Melevendi C, et al. Survival and causes of
death in thalassemia major. Lancet 1989;2:27-30.
8. Merault JM, Escamilla R, Degano B, Guittard J,
Herman C, Krempf M. Pulmonary hypertension in
patients with thalassemia major. Rev Mal Respir 2000;
17:689-91.
9. Du ZD, Roguin N, Milgram E, Saab K, Koren A. Pul-
monary hypertension in patients with thalassemia
major. Am Heart J 1997;134:532-7.
10. Derchi G, Fonti A, Forni GL, Galliera EO, Cappelini
MD, Turoti F, et al. Pulmonary hypertension in pa-
tients with thalassemia major. Am Heart J
1999;138:384
11. Alverson DC, Eldridge M, Dillon T, Yabek SM,
Berman W. Non invasive pulsed doppler determina-
tion of cardiac output in neonate and children. J
Pediatr 1982;101:46-50.
12. Ehler 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.
13. Heger JW, Roth FR, Niemann JT, Criley JM.
Echocardiography. In: Cardiology. Baltimore: William
& Wilkins; 1994. p. 93-118
14. Kulik TJ. Pulmonary hypertension. In: Fyler DC, edi-
tor. Nadas pediatric cardiology. Philladelphia: Hanley
& Belfus; 1992. p. 83-100
15. Park MK. Pulmonary hypertension. In: Pediatric car-
diology for practitioners. 3 rd ed. St Louis: Mosby;
1996. p. 420-9.
16. Park MK. Noninvasive techniques. In: Pediatric car-
diology for practitioners. 3 rd ed. St Louis: Mosby;
1996. p. 67-82.
17. Madiyono B, Moeslichan S, Sastroasmoro S, Budiman
I, Purwanto SH. Perkiraan besar sampel. In:
Sastroasmoro S, Ismael S, editors. Dasar-dasar
metodologi penelitian klinis. Jakarta: Bina Rupa
Aksara; 1995. p. 187-212
Published
2016-09-26
How to Cite
1.
Basri H, Firman A, Rusmil K, Fadlyana E. The occurrence of pulmonary hypertension in patients with thalassemia major. PI [Internet]. 26Sep.2016 [cited 26Nov.2024];43(5):162-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/686
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Received 2016-09-24
Accepted 2016-09-24
Published 2016-09-26
Accepted 2016-09-24
Published 2016-09-26
