Echocardiographic Parameters of Right Ventricular Dysfunction in Thalassemic Patients
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Published:
Feb 20, 2014
Keywords:
right ventricular function, thalassemia, pulmonary hypertension, PH
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Abstract
OBJECTIVE: To evaluate pulmonary hypertension (PH) in asymptomatic thalassemic patients using echocardiographic right ventricular (RV) parameters.
MATERIAL AND METHODS: This is a cross-sectional study. We assessed 32 ambulatory asymptomatic thalassemia cases both clinically and using echocardiographic parameters. PH was observed in 13 (40%) patients.
RESULTS:Unpaired two tail t-test between groups show significant difference in RV echocardiographic parameter S’ (p = 0.03), E’ (p = 0.016) and Tricuspid valve (TV) E/E’ (p = 0.04). Other RV echocardiographic parameters such as the right ventricular ejection fraction (RVEF), tricuspid annular plane systolic excursion (TAPSE), A, and Tei index are insignificant.
CONCLUSION: PH was found in 40% of asymptomatic thalassemic patients. Of all echocardiographic RV parameters, we found that the tissue doppler imaging (TDI): S’, E’ and TV E/E’ are the best tools for screening. This tool detects early RV dysfunction in asymptomatic thalassemias with PH.
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1.
Lekhakul A, See O, Yamwong S, Chunchrunee S. Echocardiographic Parameters of Right Ventricular Dysfunction in Thalassemic Patients. BKK Med J [Internet]. 2014 Feb. 20 [cited 2024 Nov. 23];7(1):7. Available from: https://he02.tci-thaijo.org/index.php/bkkmedj/article/view/218718
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References
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11. Du ZD, Roguin N, Milgram E, et al. Pulmonary hyper- tension in patients with thalassemia major. Am Heart J 1997;134:532-7.
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18. Grisaru D, Rachmilewitz EA, Mosseri M, et al. Cardio- pulmonary assessment in beta-thalassemia major. Chest 1990;98:1138-42.
19. Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 2001;97:3411-6.
20. Jootar P, Fucharoen S. Cardiac involvement in beta- thalassemia/hemoglobin E disease: clinical and hemo- dynamic findings. Southeast Asian J Trop Med Public Health 1990;21:269-73.
21. Aessopos A, Stamatelos G, Skoumas V, et al. Pulmonary hypertension and right heart failure in patients with beta- thalassemia intermedia. Chest 1995;107:50-3.
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23. Morris CR, et al. Pulmonary hypertension in thalassemia assessed by echocardiography: a report from baseline data of the thalassemia clinical research network Longi- tudinal Cohort Study. Blood 2009;114:796 [abst 2016].
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26. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, et al. Splenectomy: a strong risk factor for pulmonary hyper- tension in patients with thalassaemia. Heart 2006;92:1467-72.
27. Hoffbrand AV, Cohen A, Hershko C. Role of deferiprone in chelation therapy for transfusional iron overload. Blood 2003;102:17-24.
28. Aessopos A, Farmakis D, Deftereos S, et al. Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest 2005;127:1523-30.
29. Aessopos A, Farmakis D. Pulmonary hypertension in beta-thalassemia. Ann N Y Acad Sci 2005;1054:342-9.
30. Aessopos A, Farmakis D, Hatziliami A, et al. Cardiac status in well-treated patients with thalassemia major. Eur J Haematol 2004;73:359-66.
31. Kremastinos DT, Tiniakos G, Theodorakis GN, et al. Myocarditis in beta-thalassemia major. A cause of heart failure. Circulation 1995;91:66-71.
32. Buja LM, Roberts WC. Iron in the heart Etiology and clinical significance. Am J Med 1971;51:209-21.
33. Weatherall DJ. The thalassaemias. BMJ 1977;314:1675-8.
34. Olivieri NF. The beta-thalassemias. N Engl J Med 1999; 341:99-109.
35. Modell B, Khan M, Darlison M, et al. 2008. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiaovasc Magn Reson 2008;10:42.
36. Rudski LG, Lai WW, Afilalo J, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardio- graphy, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010;23:685-713.
2. Olivieri NF. The beta-thalassemias. N Engl J Med 1999;341:99-109.
3. Rund D, Rachmilewithz E. Beta-thalassemia. N Engl J Med 2005;353:1135-46.
4. Cunningham MJ, Weiss MJ, Neufeld EJ. Thalassemia In: Young NS, Gerson SL, High KA, eds. Clinical Hema- tology. Mosby Elsevier 2006:281-92.
5. Zurlo M, De Stefano P, Borgna-Pignatti C, et al. Survival and causes of death in thalassaemia major. Lancet 1989;2:27-30.
6. Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 2001;97:3411-6.
7. SonakulD, thakerngpol K, Pacharee P. Cardiac pathology in 76 thalassemic patients. Birth Defects Orig Artic Ser 1988;23:177-91.
8. Jessup M, Manno CS. Diagnosis and management of iron-induced heart disease in Colley’s anemia. Ann N Y Acad Sci 1998;850:242-50.
9. Olivieri NF, Nathan DG, MacMillan JH, et al. Survival in medically treated patients with homozygous beta-thalas- semia. N Engl J Med 1994;331:574-8.
10. Grisaru D, Rachmilewitz EA. Mosseri M. et al. Car- diopulmonary assessment in beta-thalassessmia major. Chest 1990;98:1138-42.
11. Du ZD, Roguin N, Milgram E, et al. Pulmonary hyper- tension in patients with thalassemia major. Am Heart J 1997;134:532-7.
12. Merault JM, Escamila R, Degano B, et al. Pulmonary hypertension in patients with beta-thalassemia major (in French). Rev Mal Respir 2000;17:689-91.
13. Derchi G, Fonti A, Forni GL, et al. Pulmonary hyper- tension in patients with thalassemia major. Am Heart J 1999;138:384.
14. Aessopos A, Stamatelos G, Skoumas V, et al. Pulmonary hypertension and right heart failure in patients with beta thalassemia intermedia. Chest 1995;108:50-3.
15. Koren A, Garty I, Antonelli D, et al. Right ventricular cardiac dysfunction in beta thalassemia major. Am J Dis Child 1987;141:93-6.
16. Jootar P, Fucharoen S. Cardiac involvement in beta thalas- semia/hemoglobin E disease: clinical and hemodynamic findings. Southeast Asian J rop Med Public Health 1990;21:269-73.
17. Hahalis, G. et al. Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest 2005;127:1523-30.
18. Grisaru D, Rachmilewitz EA, Mosseri M, et al. Cardio- pulmonary assessment in beta-thalassemia major. Chest 1990;98:1138-42.
19. Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 2001;97:3411-6.
20. Jootar P, Fucharoen S. Cardiac involvement in beta- thalassemia/hemoglobin E disease: clinical and hemo- dynamic findings. Southeast Asian J Trop Med Public Health 1990;21:269-73.
21. Aessopos A, Stamatelos G, Skoumas V, et al. Pulmonary hypertension and right heart failure in patients with beta- thalassemia intermedia. Chest 1995;107:50-3.
22. Singer ST, Kuypers FA, Styles L, et al. Pulmonary hyper- tension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol 2006;81:670-5.
23. Morris CR, et al. Pulmonary hypertension in thalassemia assessed by echocardiography: a report from baseline data of the thalassemia clinical research network Longi- tudinal Cohort Study. Blood 2009;114:796 [abst 2016].
24. Chuncharunee S, Atichartakarn V, Archararit N, et al. Prevalence and predictors of pulmonary arterial hyper- tension in hemoglobin E/beta-thalassemia patients [abstract]. Blood 2009;114:795.
25. Atichartkarn V, Likittanasombat K, Chuncharunee S, et al. Pulmonary arterial hypertension in previously splenec- tomized patients with beta-thalassemic disorders. Int J Hematol 2003;78:139-45.
26. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, et al. Splenectomy: a strong risk factor for pulmonary hyper- tension in patients with thalassaemia. Heart 2006;92:1467-72.
27. Hoffbrand AV, Cohen A, Hershko C. Role of deferiprone in chelation therapy for transfusional iron overload. Blood 2003;102:17-24.
28. Aessopos A, Farmakis D, Deftereos S, et al. Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest 2005;127:1523-30.
29. Aessopos A, Farmakis D. Pulmonary hypertension in beta-thalassemia. Ann N Y Acad Sci 2005;1054:342-9.
30. Aessopos A, Farmakis D, Hatziliami A, et al. Cardiac status in well-treated patients with thalassemia major. Eur J Haematol 2004;73:359-66.
31. Kremastinos DT, Tiniakos G, Theodorakis GN, et al. Myocarditis in beta-thalassemia major. A cause of heart failure. Circulation 1995;91:66-71.
32. Buja LM, Roberts WC. Iron in the heart Etiology and clinical significance. Am J Med 1971;51:209-21.
33. Weatherall DJ. The thalassaemias. BMJ 1977;314:1675-8.
34. Olivieri NF. The beta-thalassemias. N Engl J Med 1999; 341:99-109.
35. Modell B, Khan M, Darlison M, et al. 2008. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardiaovasc Magn Reson 2008;10:42.
36. Rudski LG, Lai WW, Afilalo J, et al. Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardio- graphy, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010;23:685-713.