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Cardiovascular impairment in patients with thalassemia major - is iron deposition the only factor? Comparative review of two clinical cases

Elena Marinova, Valeriya Kaleva

Abstract

Beta thalassemia is a rare hereditary disease characterized by hemolytic anemia treated with regular, monthly hemotransfusions. Patients with thalassemia develop a specific type of cardiomyopathy, which is a crucial determinant of the morbidity and mortality of the patients. Here we present two cases of patients with thalassemia major with cardiac impairment. The first case is an 18-year-old boy with sever myocardial iron deposition - the most common cause for heart failure in thalassemic patients and the second case is that of a patient with no iron deposition, normal T2* and yet with cardiac dysfunction.

Keywords

thalassemia, iron deposition, cardiovascular impairment

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References

Capellini MD, Cohen A, Porter J, Taher A, Viprakasit V, Guidelines for the treatment of transfsion dependent talassemia (TDT), 3rd edition 2014

Pennell DJ, Udelson JE, Arai AE, Bozkurt B, Cohen AR, Galanello R, Hoffman TM, Kiernan MS, Lerakis S, Piga A, Porter JB, Walker JM, Wood J; American Heart Association Committee on Heart Failure and Transplantation of the Council on Clinical Cardiology and Council on Cardiovascular Radiology and Imaging. Cardiovascular function and treatment in thalassemia major: a consensus statement from the American Heart Association. Circulation 2013;128:281-308

Kremastinos DT, Farmakis D, Aessopos A, Hahalis G, Hamodraka E, Tsiapras D, Keren A. Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives. Circ Heart Fail 2010

Farmakis D, Triposkiadis F, Lekakis J, Parissis J. Heart failure in haemoglobinopathies: pathophysiology, clinical phenotypes, and management. Eur J Heart Fail 2017; 19: 479-489

Carpenter JP, He T, Kirk P, Roughton M, Anderson LJ, de Noronha SV, Sheppard MN, Porter JB, Walker JM, Wood JC, Galanello R, Forni G, Catani G, Matta G, Fucharoen S, Fleming A, House MJ, Black G, Firmin DN, St Pierre TG, Pennell DJ.On T2* magnetic resonance and cardiac iron. Circulation 2011;123:1519-1528.

Kirk P, He T, Anderson LJ, Roughton M, Tanner MA, Lam WW, Au WY, Chu WC, Chan G, Galanello R, Matta G, Fogel M, Cohen AR, Tan RS, Chen K, Ng I, Lai A, Fucharoen S, Laothamata J, Chuncharunee S, Jongjirasiri S, Firmin DN, Smith GC, Pennell DJ International reproducibility of single breathhold T2* MR for cardiac and liver iron assessment among five thalassemia centers. J Magn Reson Imaging. 2010;32:315-319

Alpendurada F, Carpenter JP, Deac M, Kirk P, Walker JM, Porter JB, Banya W, He T, Smith GC, Pennell DJ Relation of myocardial T2* to right ventricular function in thalassaemia major. Eur Heart J. 2010;31:1648-1654

Tanner MA, Galanello R, Dessi C, Smith GC, Westwood MA, Agus A, Roughton M, Assomull R, Nair SV, Walker JM, Pennell DJ A randomized, placebo-controlled, double-blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance. Circulation. 2007;115:1876-1884

Papakonstantinou O, Alexopoulou E, Economopoulos N, Benekos O, Kattamis A, et al. Assessment of iron distribution between liver, spleen, pancreas, bone marrow, and myocardium by means of R2 relaxometry with MRI in patients with beta-thalassemia major. J Magn Reson Imaging 2009; 29: 853-859.

Farmakis D, Aessopos A. Pulmonary hypertension associated with hemoglobinopathies: prevalent but overlooked. Circulation 2011;123:1227-1232.

Димова М., Каназирев Б., Златева В., Калева В., Герчева Св., Петрова Кр., Епидемиология и патофизиология на сърдечно-съдовото засягане при пациенти с бета-таласемия майор, Наука Кардиология, брой 2, 2017

Kanazirev B, Dimova M, Kaleva V, Gercheva S, Petrova K, Zlateva V, et al, Early identification of heart failure in patients with thalassemia major by NT-pro-BNP examination. Correlation with echocardiographic parameters of morphology and function, International Journal of Medical and Health Research, vol3, iss. 12, 2017

Димова М., Каназирев Б., Златева В., Калева В., Герчева Св., Петрова Кр. Бета-Таласемия и маркери за ранно сърдечно-съдово засягане, Наука Кардиология , брой 3, 2017

AndersonLJ,WestwoodMA,HoldenS, et al. Myocardial iron clearance during reversal of siderotic cardiomyopathy with intravenous desferrioxamine: A prospective study using T2* cardiovascular magnetic resonance. Br J Haematol 2004 ,348–355

Aessopos А , Farmakis D,⁎, Aikaterini Polonifi a, Maria Tsironi a, Christina Fragodimitri b, Antonia Hatziliami b, Markisia Karagiorga b,Evanthia Diamanti-Kandarakis, Plasma B-type natriuretic peptide concentration in β-thalassaemia patients,Eur J Heart Fail. 2007 May;9(5):537-41

. Димова М., Каназирев Б., Златева В., Калева В., Герчева Св., Петрова Кр., Николова С., Ранна диагноза на левокамерна дисфункция при пациенти с бета-Таласемия Майор чрез тъканен Доплер, стрейн и стрейн рейт , Наука Кардиология , брой 4, 2017год.

Димова М, Каназирев Б, Калева В, Герчева С, Петрова К, Златева В, Бочева Я, Николова С, Показатели на диастолна функция и биохимични показатели – сравнение на пациенти с Таласемия Майор и здрави контроли, сп. Българска Кардиология бр. 1/2018 (45-50)

Пенчо Георгиев, Катя Сапунарова, Веселина Горанова-Маринова, Бета- таласемия майор , железен свръхтовар и хелатиране – съвременни тенденции. Сп. Хематология 1-2/2017 (25-32стр.)

Stoyanova E, Cloutier G, Felfly H, Lemsaddek W, Ah-Son N, Trudel M. Evidence for a novel mechanism independent of myocardial iron in β-thalassemia cardiac pathogenesis. PLoS One 2012;7(12):e52128

Georgieva Z, Georgieva M. Compensatory and adaptive changes in microcirculation and left ventricular function of patients with chronic iron-deficiency anaemia. Clin Hemorheol Microcirc 1997;17:21–30.

Cho IJ et al, Effect of anemia correction on left ventricular structure and filling pressure in anemic patients without overt heart disease. Korean J Intern Med. 2014;29(4):445-53.

Zhou Q,, Shen J, Liu Y, Luo R, Tan B, Li G, Assessment of left ventricular systolic function in patients with iron deficiency anemia by three-dimensional speckle-tracking echocardiography., Anatol J Cardiol. 2017 Sep;18(3):194-199

Shen J et al. Evaluation of left atrial function in patients with iron-deficiency anemia by two-dimensional speckle tracking echocardiography. Cardiovasc Ultrasound.2016;14(1):34.

Димова М, Каназирев Б, Калева В, Герчева С, Петрова К, Златева В, Бочева Я, Николова С, Хемодинамични и ехокардиографски показатели на систолна функция при пациенти с Таласемия майор в сравнение с контроли., сп. Българска Кардиология бр. 1/2018 (40-44)




DOI: http://dx.doi.org/10.14748/vmf.v8i1.5789

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