Left ventricular functions and mass of the adolescents and young adults with thalassemia major: An echocardiography study
Keywords:
thalassemia major, left ventricular functions and mass, ferritin serum concentration
Abstract
Background Thalassemia major (TM) is a transfusion-depen-dent disease. Multiple transfusions cause an accumulation of iron
in the body. On the heart, iron can induce cardiomyopathy, the
leading cause of death in TM.
Objective To evaluate left ventricular functions and mass among
adolescents and young adults with TM.
Methods A cross-sectional study was conducted on 58 patients
with TM in Cipto Mangunkusumo Hospital, Jakarta. Hematological
data was collected from medical record. Left ventricular systolic func-
tions (EF, FS), diastolic functions (A, E, E/A ratio, IVRT), and mass
(LVDDi, LVDSi, LVMi) were detected using an echocardiography
Sonos 4500. T test, linier regression, and multiple regression analy-
sis were performed.
Results Out of 32 TM patients, 30 were enrolled to study. Left
ventricular systolic and diastolic functions of TM patients were lower
than those of the control and statistically significant [EF 66.1% (SD
4.9) and 71.6% (SD 5.6); P<0.0001, FS 36.0% (SD 3.7) and 39.8%
(SD 5.5); P=0.003, E/A 2.14 (SD 0.4) and 1.83 (SD 0.3); P=0.002,
respectively]. Left ventricular mass of TM patients was greater than
that of control, and also statistically significant [LVMi 111.1 g/m 2
(SD 30.8) and 75.4 g/m 2 (SD 14.5); P<0.0001, respectively]. Linier
and multiple regression analysis showed significant and powerful
correlation between left ventricular diastolic functions (E/A ratio)
and serum ferritin concentration (r=0.71; P<0.0001).
Conclusion Left ventricular functions and mass of adolescents
and young adults with TM show abnormalities. The higher the con-
centration of ferritin serum is, the more likely for TM patient to suf-
fer from diastolic abnormality
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thalassaemia major: A Doppler echocardiographic as-
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mensional, and Doppler echocardiographic study. Am
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GG, Goldberg SJ, Allen HD, et al. Preclinic abnormal
segment cardiac manifestations of thalassemia major in
children on transfusion-chelation therapy; echographic
alteration of left ventricular posterior wall contraction
and relaxation patterns. Am Heart J 1982;103:505-11.
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cardiac dysfunction in patients with severe β-
thalassaemia and chronic iron overload. N Engl J Med
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lation 1982;66:980-5.
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Lafair JS, Okon E, et al. Cardiopulmonary assessment
in beta thalassaemia major. Chest 1990;98:1138-42.
24. Appleton CP, Hatle LK, Popp RL. Demonstration of
restrictive ventricular physiology by Doppler echo-
cardiography. J Am Coll Cardiol 1988;11:757-68.
25. Cheung YF, Chan GCF, Ha SY. Arterial stiffness and
endothelial function in patients with â–thalassemia
major. Circulation 2002;106:2561-6.
26. Lattanzi F, Belloti P, Picano E, Chiarella F, Mazzarisi A,
Melevendi C, et al. Quantitative ultrasonic analysis of
myocardium in patients with thalassemia major and
iron overload. Circulation 1993;87;748-54.
27. Kremastinos DT, Rentoukaas E, Mavrogeni S. Left ven-
tricular inflow pattern in â-thalassemia major: A Doppler
echocardiographic study. Eur Heart J 1993;14:351-7.
28. Henry WL, Nienhuis WA, Wiener M, Miller R, Canale
VC, Piomelli S. Echocardiographic abnormalities in
patients with transfusion-dependent anemia and sec-
ondary myocardial iron deposition. Am J Med
1978;64:547-55.
29. Aldouri MA, Wonke B, Hoffbrand AV, Flynn DM,
Ward SE, Agnew JE, Hilson AJ. High incidence of car-
diomyopathy in beta-thalassaemia patients receiving
regular transfusion and iron chelation: Reversal by in-
tensified chelation. Acta Haematol 1990; 84;113-7.
30. Pennell DJ. Iron overload and the heart. Hematology
2004;2004:20-9.
31. Politi A, Sticca M, Galli M. Reversal of haemochromatotic
cardiomyopathy in beta thalassaemia by chelation therapy.
British Heart Journal 1995;73:486-7.
32. Piga A, Gaglioti C, Fogliocco E, Tricta F. Compara-
tive effects of deferiprone and deferoxamine on sur-
vival and cardiac disease in patients with thalassemia
major: A retrospective analysis. Haematologica 2003;
88(05):489-96.
Loutradi A, Hatziliami A, et al. Cardiac involvement
in thalassemia intermedia: A multicenter study. Blood
2001;97:3411-6.
2. Gibbons R, Higgs DR, Olivieri NF, Wood WG. The â-
thalassemias. In: Weatherall DJ, Clegg JB, editors. The
thalassemia syndrome. 4th ed. London: Blackwell Sci-
ence; 2001. p. 287-300.
3. Honig GR. Hemoglobin disorders. In: Behrman RE,
Kliegman RM, Jenson HB, editors. Nelson textbook of
pediatrics. 16th ed. Philadelphia: W.B. Saunders Com-
pany; 2000. p. 1484-5.
4. Borgna-Pignatti C, Rugolotto S, De Stepano P, Piga A,
Di Gregorio F, Gamberini MR, et al. Survival and dis-
ease complications in thalassemia major. Ann NY Acad
Sci 1998;850:227-31.
5. Jessup M, Manno CS. Diagnosis and management of
iron-induced heart disease in cooley’s anemia. Ann NY
Acad Sci 1998;850:242-50.
6. Caddel JL. Metabolic and nutritional diseases and dis-
eases. In: Allen HD, Gutgessel HP, Clark EB, Driscoll
DJ, editors. Moss and Adam heart disease in infants,
children, and adolescents. 6th ed. Philadelphia:
Lippincott Williams & Wilkins; 2001. p. 1263-4.
7. Olivieri NF. The β-thalassemias. N Engl J Med
1999;341:99-109.
8. Kremastinos DT, Tsiapras DP, Tsetsos GA, Rentoukas
EI, Vretou HP, Toutouzas PK. Left ventricular dias-
tolic Doppler characteristics in â-thalassaemia major.
Circulation 1993;88:1127-35.
9. Spirito P, Lupi G, Melevendi C, Vecchio C. Restric-
tive diastolic abnormalities identified by Doppler
echocardiography in patients with thalassemia ma-
jor. Circulation 1990;82:88-94.
10. Hoffband AV. A sensitive test for early myocardial load-
ing. Eur Heart J 2003;24:26-7.
11. Vaccari M, Crepaz R, Fortini M, Gamberini MR,
Scarcia S, Pitscheider W, et al. Left ventricular re-
modeling, systolic function, and diastolic function in
young adults with β –thalassemia intermedia. Chest
2002;121:506-12.
12. Bosi G, Crepaz R, Gamberini MR, Fortini M, Scarcia
S, Bonsante E, et al. Left ventricular remodeling, and
systolic and diastolic function in young adults with â-
thalassaemia major: A Doppler echocardiographic as-
sessment and correlation with hematological data.
Heart 2003;89:762-6.
13. Kremastinos DT, Tiniakos G, Theodorakis GN,
Katritsis DG, Toutouzas PK. Myocarditis in â-thalas-
semia major. Circulation 1995;91:66-71.
14. Metivier F, Marchais SJ, Guerin AP, Pannier B, Lon-
don GM. Pathophysiology of anaemia: Focus on the
heart and blood vessels. Nephrol dial transplant
2000;15(suppl 3):14-8.
15. Varat MA, Adolph RJ, Fowler NO. Cardiovascular ef-
fect of anemia. Am Heart J 1972;83:415-26.
16. Olivieri NF, Nathan DG, MacMillan JH, Wayne AS,
Liu PP, McGee A, et al. Survival in medically treated
patients with homozygous β-thalassemia. N Engl J Med
1994;331:574-8.
17. Olivieri NF, Brittenham GM. Iron-chelating therapy
and the treatment of thalassemia. Blood 1997;89:739-
61.
18. Lau KC, Li AMC, Hui PW, Yeung CY. Left ventricular
function in β-thalassaemia major. Arch Dis Child
1989;64:1046-51.
19. Bhal VK, Malhotra OP, Kumar D, Agarwal R, Goswami
KC, Bajaj R, et al. Non invasive assessment of systolic
and diastolic left ventricular function in patients with
chronic severe anemia: A combined m-mode, two-di-
mensional, and Doppler echocardiographic study. Am
Heart J 1992;124:1516-23.
20. Valdes-Cruz LM, Reinecke C, Rutkowski M, Dudell
GG, Goldberg SJ, Allen HD, et al. Preclinic abnormal
segment cardiac manifestations of thalassemia major in
children on transfusion-chelation therapy; echographic
alteration of left ventricular posterior wall contraction
and relaxation patterns. Am Heart J 1982;103:505-11.
21. Leon MB, Borer JS, Bacharach SL. Detection of early
cardiac dysfunction in patients with severe β-
thalassaemia and chronic iron overload. N Engl J Med
1979;301:1143-8.
22. Borow KM, Propper R, Bierman FZ, Grady S, Inati A.
The left ventricular end-systolic pressure-dimension
relation in patients with thalassemia major. A new non-
invasive method for assessing contractile state. Circu-
lation 1982;66:980-5.
23. Grisaru D, Rachmilewitz EA, Mosseri M, Gostman M,
Lafair JS, Okon E, et al. Cardiopulmonary assessment
in beta thalassaemia major. Chest 1990;98:1138-42.
24. Appleton CP, Hatle LK, Popp RL. Demonstration of
restrictive ventricular physiology by Doppler echo-
cardiography. J Am Coll Cardiol 1988;11:757-68.
25. Cheung YF, Chan GCF, Ha SY. Arterial stiffness and
endothelial function in patients with â–thalassemia
major. Circulation 2002;106:2561-6.
26. Lattanzi F, Belloti P, Picano E, Chiarella F, Mazzarisi A,
Melevendi C, et al. Quantitative ultrasonic analysis of
myocardium in patients with thalassemia major and
iron overload. Circulation 1993;87;748-54.
27. Kremastinos DT, Rentoukaas E, Mavrogeni S. Left ven-
tricular inflow pattern in â-thalassemia major: A Doppler
echocardiographic study. Eur Heart J 1993;14:351-7.
28. Henry WL, Nienhuis WA, Wiener M, Miller R, Canale
VC, Piomelli S. Echocardiographic abnormalities in
patients with transfusion-dependent anemia and sec-
ondary myocardial iron deposition. Am J Med
1978;64:547-55.
29. Aldouri MA, Wonke B, Hoffbrand AV, Flynn DM,
Ward SE, Agnew JE, Hilson AJ. High incidence of car-
diomyopathy in beta-thalassaemia patients receiving
regular transfusion and iron chelation: Reversal by in-
tensified chelation. Acta Haematol 1990; 84;113-7.
30. Pennell DJ. Iron overload and the heart. Hematology
2004;2004:20-9.
31. Politi A, Sticca M, Galli M. Reversal of haemochromatotic
cardiomyopathy in beta thalassaemia by chelation therapy.
British Heart Journal 1995;73:486-7.
32. Piga A, Gaglioti C, Fogliocco E, Tricta F. Compara-
tive effects of deferiprone and deferoxamine on sur-
vival and cardiac disease in patients with thalassemia
major: A retrospective analysis. Haematologica 2003;
88(05):489-96.
Published
2016-10-18
How to Cite
1.
Ali M, Putra S, Gatot D, Sastroasmoro S. Left ventricular functions and mass of the adolescents and young adults with thalassemia major: An echocardiography study. PI [Internet]. 18Oct.2016 [cited 23Dec.2024];46(5):214-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/932
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Received 2016-10-16
Accepted 2016-10-16
Published 2016-10-18
Accepted 2016-10-16
Published 2016-10-18
