Abstract
Splenomegaly is common in β-thalassemia, bearing some particular hemodynamic features, while splenectomy is an established therapeutic intervention in these patients. Their effects, however, on systemic hemodynamics and thalassemia heart disease have not yet been assessed. The study included 32 consecutive patients, 13 with thalassemia major (TM) and 19 with thalassemia intermedia (TI), aged 23.4±4.2 years, requiring splenectomy. Assessment was performed before and 6 months after splenectomy and included hematological profile and resting echocardiography; total blood volume was also measured in 10 of 32 cases. Preoperative echocardiographic data were compared with those of 34 controls. Preoperative left ventricular diameters and mass, cardiac index, and systolic pulmonary artery pressure were all significantly higher in patients compared to controls (p<0.001), but did not differ significantly between TM and TI patients. Postoperatively, the mean hemoglobin level increased from 8.1±0.6 to 9.1±0.4 g/dl (p<0.001), total blood volume index declined from 2847±332 to 2310±276 ml/m2 (p<0.001), blood transfusions were discontinued in 80% of TI patients and mean 6-monthly transfusion requirements in TM patients were reduced from 28±5 to 22±4 units (p<0.001). However, cardiac parameters were not significantly modified. It seems that left ventricular remodeling, high output state, and increased pulmonary artery pressure characterize both TM and TI patients who require splenectomy. Although these abnormalities remain unchanged after splenectomy, the removal of the spleen may contribute to the prevention of further cardiac damage by ameliorating the patients’ hematological status and reducing their transfusion needs.
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Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A, Di Gregorio F, Gamberini MR, Sabato V, Melevendi C, Cappellini MD, Verlato G (1998) Survival and disease complications in thalassemia major. Ann N Y Acad Sci 850:227–231
Jessup M, Manno CS (1998) Diagnosis and management of iron-induced heart disease in Cooley’s anemia. Ann N Y Acad Sci 850:242–250
Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, Joussef J, Rombos J, Loukopoulos D (2001) Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 97:3411–3416
Olivieri NF (1999) The beta-thalassemias. N Engl J Med 341:99–109
Wonke B (2001) Clinical management of β-thalassemia major. Semin Hematol 38:350–359
Graziano G, Piomelli S, Hilgartner M, Giardina P, Karpatkin M, Andrew M, Lolacono N, Seaman C (1981) Chelation therapy in thalassemia major. III. The role of splenectomy in achieving iron balance. J Pediatr 99:695–699
Sahn DJ, De Maria A, Kisslo J, Weyman A (1978) The committee on M-mode standardization of the American Society of Echocardiography: results of a survey of echocardiographic measurements. Circulation 58:1072–1083
Devereux RB, Alonso DR, Lutas EM, Gottlieb GJ, Campo E, Sachs I, Reichek N (1986) Echocardiographic assessment of left ventricular hypertrophy: comparison to necropsy findings. Am J Cardiol 57:450–458
Schiller NB, Shah PM, Crawford M, DeMaria A, Devereux R, Feigenbaum H, Gutgessel H, Reicheh N, Sahn D, Schnittger I, Silverman NH, Tajih AJ (1989) Recommendations for quantitation of the left ventricle by two-dimensional echocardiography: American Society of Echocardiography Committee on Standards Subcommittee. J Am Soc Echocardiogr 2:358–367
Aessopos A, Farmakis D, Taktikou H, Loukopoulos D (2000) Doppler-determined peak systolic tricuspid pressure gradient in persons with normal pulmonary function and tricuspid regurgitation. J Am Soc Echocardiogr 13:645–649
Aessopos A, Farmakis D, Tsironi M, Deftereos S, Tassiopoulos S, Konstantopoulos K, Rombos J, Papalambros E (2004) Hemodynamic assessment of splenomegaly in beta-thalassemia patients undergoing splenectomy. Ann Hematol 83:775–778
Engelhard D, Gividalli G, Rachmilewitz EA (1975) Splenectomy in homozygous beta thalassaemia: a retrospective study of 30 patients. Br J Haematol 31:391–403
Blendis LM, Modell CB, Bowdler AJ, Williams R (1974) Some effects of splenectomy in thalassaemia major. Br J Haematol 28:77–87
Bahl VK, Malhotra OP, Kumar D, Agarwal R, Goswami KC, Bajaj R, Shrivastava S (1992) Non-invasive assessment of systolic and diastolic left ventricular function in patients with chronic severe anemia: a combined M-mode, two-dimensional, and Doppler echocardiographic study. Am Heart J 124:1516–1523
Varat MA, Adolph RJ, Fowler NO (1972) Cardiovascular effects of anemia. Am Heart J 83:415–426
Garnett ES, Goddard BA, Markby D, Webber CE (1969) The spleen as an arteriovenous shunt. Lancet 1:386–388
Konstantopoulos K, Plataniotis G, Maris T, Voskaridou E, Rekoumi L, Stratigaki M, Loukopoulos D (1995) Extramedullary haemopoiesis in thalassaemia intermedia: an unusual case of relapsing paraparesis in pregnancy. Haematologia (Budap) 27:29–32
Braunwald E (2001) Heart disease, 6th edn. W.B. Saunders, Philadelphia, pp 2226–2227
Sonakul D, Fucharoen S (1992) Pulmonary thromboembolism in thalassemic patients. Southeast Asian J Trop Med Public Health 23 [Suppl 2]:25–28
Aessopos A, Farmakis D, Hatziliami A, Fragodimitri C, Karabatsos F, Joussef J, Mitilineoy E, Diamanti-Kandaraki E, Meletis J, Karagiorga M (2004) Cardiac status in well-treated patients with thalassemia major. Eur J Haematol 73:359–366
Du ZD, Roguin N, Milgram E, Saab K, Koren A (1997) Pulmonary hypertension in patients with thalassemia major. Am Heart J 134:532–537
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Aessopos, A., Farmakis, D., Deftereos, S. et al. Cardiovascular effects of splenomegaly and splenectomy in β-thalassemia. Ann Hematol 84, 353–357 (2005). https://doi.org/10.1007/s00277-004-1002-4
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DOI: https://doi.org/10.1007/s00277-004-1002-4