Published: 2019-12-25

Left ventricular dysfunction by strain echocardiography in thalassemia patients: a pilot study

Akshay Ashok Bafna, Hetan C. Shah


Background: To evaluate the myocardial function and its correlation with serum ferritin and the number of transfusions in beta-thalassemia major patients by using standard echocardiography and left ventricular strain imaging.

Methods: This was a cross-sectional exploration study comprised of 56 beta-thalassemia patients conducted at a tertiary-care center in India between September 2016 and August 2017. Patients with age less than 18 years, diagnosed with thalassemia major, recipients of >20 units of blood transfusions, and normal Left Ventricular (LV) function by 2D-echocardiography were included in the study. Severity of iron overload was determined by using serum ferritin levels and LV strain imaging parameters were evaluated by using strain values of 17 LV segments.

Results: A total of 56 beta-thalassemia patients were included in the study. Of these, 29(51.8%) patients were boys and 27(48.2%) patients were girls with a mean age of 7.8±1.84 years. Average serum ferritin level was found to be 4089.83 ng/dl. Strain values of the basal lateral wall of the left ventricle were significantly abnormal in patients who received more (>80) transfusions compared with those who received lesser transfusions (p=0.025 and p=0.045), respectively. Patients with serum ferritin >6000 ng/ml had impaired strain (p=0.03).

Conclusions: Conventional echocardiographic parameters and Left Ventricular Ejection Fraction (LVEF) do not provide adequate information about LV dysfunction. Systolic strain index imaging of the LV indicated the presence of early LV systolic dysfunction in patients who received a greater number of blood transfusions and patients with higher serum ferritin levels.


Beta-thalassemia, Blood transfusion, Echocardiography, Heart ventricles, Iron overload

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Parsaee M, Saedi S, Joghataei P, Azarkeivan A, Alizadeh Sani Z. Value of speckle tracking echocardiography for detection of clinically silent left ventricular dysfunction in patients with β-thalassemia. Hematol. 2017 Oct 21;22(9):554-8.

Rund D, Rachmilewitz E. β-Thalassemia. New Engl J Med. 2005 Sep 15;353(11):1135-46.

Camaschella C, Cappellini MD. Thalassemia intermedia. Haematol. 1995 Jan 1;80(1):58-68.

Aessopos A, Berdoukas V, Tsironi M. The heart in transfusion dependent homozygous thalassaemia today–prediction, prevention and management. Euro J Haematol. 2008 Feb;80(2):93-106.

Tubman VN, Fung EB, Vogiatzi M, Thompson AA, Rogers ZR, Neufeld EJ, et al. Guidelines for the standard monitoring of patients with thalassemia: report of the thalassemia longitudinal cohort. J Pediatr Hematol/Oncol. 2015 Apr;37(3):e162.

Bilge AK, Altinkaya E, Ozben B, Pekun F, Adalet K, Yavuz S. Early detection of left ventricular dysfunction with strain imaging in thalassemia patients. Clin Cardiol. 2010 Jul;33(7):E29-34.

Peng CT, Chang JS, Wu KH, Tsai CH, Lin HS. Mechanisms of and obstacles to iron cardiomyopathy in thalassemia. Front Biosci. 2008 May 1;13(1):5975-87.

Anderson LJ, Wonke B, Prescott E, Holden S, Walker JM, Pennell DJ. Comparison of effects of oral deferiprone and subcutaneous desferrioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia. Lancet. 2002 Aug 17;360(9332):516-20.

Glickstein H, El RB, Link G, Breuer W, Konijn AM, Hershko C, et al. Action of chelators in iron-loaded cardiac cells: accessibility to intracellular labile iron and functional consequences. Blood. 2006 Nov 1;108(9):3195-203.

Kim E, Giri SN, Pessah IN. Iron (II) is a modulator of ryanodine-sensitive calcium channels of cardiac muscle sarcoplasmic reticulum. Toxicol Appl Pharmacol. 1995 Jan 1;130(1):57-66.

Rodrigues A, Guimarães-Filho FV, Braga JC, Rodrigues CS, Waib P, Fabron-Junior A, et al. Echocardiography in thalassemic patients on blood transfusions and chelation without heart failure. Arquiv Brasilei Cardiol. 2013 Jan;100(1):75-81.

Vogel M, Anderson LJ, Holden S, Deanfield JE, Pennell DJ, Walker JM. Tissue Doppler echocardiography in patients with thalassaemia detects early myocardial dysfunction related to myocardial iron overload. Euro Heart J. 2003 Jan 1;24(1):113-9.

Aypar E, Alehan D, Hazırolan T, Gümrük F. The efficacy of tissue Doppler imaging in predicting myocardial iron load in patients with beta-thalassemia major: correlation with T2* cardiovascular magnetic resonance. Inter J Cardio Imag. 2010 Apr 1;26(4):413-21.

Weidemann F, Strotmann JM. Use of tissue Doppler imaging to identify and manage systemic diseases. Clin Res Cardiol. 2008 Feb 1;97(2):65-73.

Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. J Cardio Mag Reson. 2008 Dec;10(1):42.

Kremastinos DT, Tsetsos GA, Tsiapras DP, Karavolias GK, Ladis VA, Kattamis CA. Heart failure in beta thalassemia: a 5-year follow-up study. Am J Med. 2001 Oct 1;111(5):349-54.

Hamdy AM. Use of strain and tissue velocity imaging for early detection of regional myocardial dysfunction in patients with beta thalassemia. Euro J Echocar. 2007 Mar 1;8(2):102-9.

Magrì D, Sciomer S, Fedele F, Gualdi G, Casciani E, Pugliese P, et al. Early impairment of myocardial function in young patients with β‐thalassemia major. Euro J Haematol. 2008 Jun;80(6):515-22.

Perk G, Tunick PA, Kronzon I. Non-Doppler two-dimensional strain imaging by echocardiography–from technical considerations to clinical applications. J Am Soc Echocardio. 2007 Mar 1;20(3):234-43.

Nesbitt GC, Mankad S. Strain and strain rate imaging in cardiomyopathy. Echocardio. 2009 Mar;26(3):337-44.