Abstract
Heart complications are among the serious problems of patients with β-thalassemia intermedia. This study aimed to evaluate myocardial function in these patients. Clinical parameters and both standard Doppler and pulsed Doppler tissue imaging parameters were compared in 51 β-thalassemia intermedia patients (mean age, 17.05 ± 5.8 years) and 20 normal subjects (mean age, 17.81 ± 7.35 years, p = 0.98). In 11 patients (21.5%), pulmonary artery hypertension was detected. M-mode echocardiographic findings such as ejection fraction and fractional shortening did not show statistically significant changes (p > 0.005). Pulsed Doppler showed a significant difference in the early (E) to late diastolic (A) velocity ratio of the tricuspid and mitral valve between the patients and the control subjects (p < 0.05). In the pulsed tissue Doppler study, the peak systolic velocity of the septum (Ss), the peak atrial velocity of the septum (Aas), the peak systolic velocity of the tricuspid annulus (St), the peak early diastolic velocity of the tricuspid annulus (Eat), and the peak late diastolic velocity of the tricuspid annulus (Aat) were increased significantly (p < 0.05). The pulse tissue Doppler of the lateral mitral annulus did not change significantly (p > 0.005). The peak systolic velocity of the posterior wall and the peak late diastolic velocity of the anterior wall changed significantly (p < 0.05). This study showed that β-thalassemia intermedia patients with normal M-mode and two-dimensional echocardiography had statistically significant changes in pulsed Doppler and pulsed tissue Doppler imaging.
Similar content being viewed by others
References
Aessopos A, Farmakis D (2005) Pulmonary hypertension in beta-thalassemia. Ann NY Acad Sci 1054:342–349
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
Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, Karagiorga M (2005) Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest 127:1523–1530
Aessopos A, Kati M, Farmakis D (2007) Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica 92:658–665
Bosi G, Crepaz R, Gamberini MR, Fortini M, Scarcia S, Bonsanate E, Pitscheider W, Vaccari M (2003) Left ventricular remodeling and systolic and diastolic function in young adults with β-thalassemia major: a Doppler echocardiographic assessment and correlation with haematological data. Heart 89:762–766
Camaschella C, Capellini MD (1995) Thalassemia intermedia. Haematologica 80:58–68
Derchi G, Forni GL, Formisano F, Cappellini MD, Galanello R, D’Ascola G, Bina P, Magnano C, Lamagna M (2005) Efficacy and safety of sildenafil in the treatment of severe pulmonary hypertension in patients with hemoglobinopathies. Haematologica 90:452–458
Forget BG (2000) Thalassemia syndromes. In: Hoffman R, Benz EJ Jr, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, McGlave P (eds) Hematology: basic principles and practice. Churchill Livingstone, New York, p 485
Gorcsan J, Gulati VK, Mandarino WA, Katz WE A (1996) Color-coded measures of myocardial velocity throughout the cardiac cycle by tissue Doppler imaging to quantify regional left ventricular function. Am Heart J 131:1203–1213
Iarussi D, Di Salvo G, Pergola V, Coppolino P, Tedesco MA, Ratti G, Esposito L, Calabrò R (2003) Pulsed tissue imaging and myocardial function in thalassemia major. Heart vessels 18:1–6
Isma’eel H, El Chafic AH, El Rassi F, Inati A, Koussa S, Daher R, Gharzuddin W, Alam S, Taher A (2003) Relation between iron overload indices, cardiac echo-Doppler, and biochemical markers in thalassemia intermedia. Am J Cardiol 102:363–367
Leon MB, Borer JS, Bacharach SL, Green MV, Benz EJ Jr, Griffith P, Nienhuis AW (1979) Detection of early cardiac dysfunction in patients with severe thalassemia and chronic iron overload. N Engl J Med 301:1143–1148
Miyatake K, Yamagishi M, Tanaka N, Uematsu M, Yamazaki N, Mine Y, Sano A, Hirama M (1995) New method of evaluating left ventricular wall motion by color-coded tissue Doppler imaging: in vitro and in vivo studies. J Am Coll Cardiol 25:717–724
Morris CR, Kuypers FA, Kato GJ, Lavrisha L, Larkin S, Singer T et al (2005) Hemolysis-associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci 1054:481–485
Nagueh SF, Middleton KJ, Kopelen HA, Zoghbi WA, Quinones MA (1997) Doppler tissue imaging: a noninvasive technique for evaluation of left ventricular relaxation and estimation of filling pressures. J Am Coll Cardiol 30:1527–1533
Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A, Martin M, Koren G, Cohen AR (1994) Survival in medically treated patients with homozygous β-thalassemia. N Engl J Med 331:574–578
Palka P, Lange A, Fleming AD, Sutherland GR (1995) Doppler tissue imaging: myocardial wall motion velocities in normal subjects. J Am Soc Echocardiogr 8:659–668
Pellerin D, Cohen L, Larrazet F, Pajany F, Witchitz S, Veyrat C (1997) Preejectional left ventricular wall motion in normal subjects using Doppler tissue imaging and correlation with ejection fraction. Am J Cardiol 80:601–607
Rashidighader F, Vahidshahi K (2008) Evaluation of the pulmonary hypertension in thalassemia intermedia. Res J Biol Sci 3:794–797
Safar ME, Levy BI, Struijker-Boudier H (2003) Current perspectives on arterial stiffness and pulse pressure in hypertension and cardiovascular diseases. Circulation 107:2864–2869
Silvilairat S, Sittiwangkul R, Pongprot Y, Charoenkwan P, Phornphutkul C (2008) Tissue Doppler echocardiography reliably reflects severity of iron overload in pediatric patients with β thalassemia. Eur J Echocardiogr 9:368–372
Singer ST, Kuypers FA, Styles L, Vichinshky EP, Foote D, Rosenfeld H (2006) Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol 81:670–675
Sutherland GR, Hatle L (2000) Pulsed Doppler myocardial imaging: a new approach to regional longitudinal function? Eur J Echocardiogr 1:81–83
Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned MS, El-Chafic AH1, Fasulo MR, Cappellini MD (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 115:1886–1892
Vaccari M, Crepaz R, Fortini M, Gamberini MR, Scarcia S, Pitscheider W, Bosi G (2002) Left ventricular remodeling, systolic function, and diastolic function in young adults with β-thalassemia intermedia. Chest 121:506–512
Valdes Cruz LM, Reinecke C, Rutkowski M, Dudell GG, Goldberg SJ, Allen HD, Sahn DJ, Piomelli S (1982) Preclinical abnormal segmental cardiac manifestations of thalassemia major in children on transfusion-chelation therapy: echocardiographic alterations of left ventricular posterior wall contraction and relaxation pattern. Am Heart J 103:505–511
Acknowledgments
This work was financially supported by the office of the Vice Chancellor for Research of Shiraz University of Medical Sciences. We thank K. Shashok (author AID in the Eastern Mediterranean) for improving the use of English in the manuscript.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Amoozgar, H., Farhani, N. & Karimi, M. Early Echocardiographic Findings in β-Thalassemia Intermedia Patients Using Standard and Tissue Doppler Methods. Pediatr Cardiol 32, 154–159 (2011). https://doi.org/10.1007/s00246-010-9834-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-010-9834-2