Assessment of Cardiac Functions and Arrhythmia in Children with Beta-Thalassemia Major and Beta-Thalassemia Intermedia

Authors

  • Khaled Salama Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
  • Hanan Zekri Khaled Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
  • Hadeel Mohamed Seif El Dien Department of Radiodiagnosis, Faculty of Medicine, Cairo University, Cairo, Egypt
  • Rasha Abdel-Raouf Abdel-Aziz Afifi Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt
  • Naglaa Mohamed Mahmoud Shaheen Department of Hematology, Ministry of Health, Atfal Masr Hospital, Health Insurance Organization, Cairo, Egypt
  • Mehan Abdalla Mohamed Department of Hematology, Ministry of Health, Atfal Masr Hospital, Health Insurance Organization, Cairo, Egypt https://orcid.org/0000-0001-7200-8018

DOI:

https://doi.org/10.3889/oamjms.2022.9026

Keywords:

Thalassemia, Echocardiography, Arrhythmia, Cardiac MRIT2*(CMRIT2*)

Abstract

BACKGROUND: Heart disease is a major complication in thalassemic patients. Heart injuries in iron overload cases include arrhythmia, pulmonary hypertension, systolic/diastolic dysfunction, and heart failure.

AIM: This study aimed to assess cardiac functions and arrhythmia in children with β-thalassemia major (TM) and β-thalassemia intermedia (TI) and its relation to cardiac iron overload.

METHODS: Thirty β-TM patients and 30 β-TI patients were evaluated using echocardiography and 24-h ambulatory electrocardiogram monitoring (Holter). Among these patients, 15 β-TM and 15 β-TI patients were evaluated using cardiac magnetic resonance imaging T2* by single breath-hold multi-echo technique.

RESULTS: Arrhythmia was detected significantly more in β-TM patients than β-TI (p = 0.049). Nine (30%) β-TM and five (16.6%) β-TI patients had Sinus tachycardia. Two (6.7%) β-TM patients compared to one (3.33%) β-TI patient had supraventricular tachycardia runs. Three (10%) β-TM and one (3.33%) β-TI patient had extreme sinus tachycardia. Two (3.3%) β-TI patients had sinus bradycardia, while two (3.3%) β-TM patients had incomplete Right bundle branch block. Regarding echo parameters: Isovolumic relaxation time (IVRT), Left ventricle myocardial performance index (MPI LV), Right ventricle myocardial performance index (MPI RV) and end systolic pulmonary artery pressure, were significantly higher in β-TM than TI group (p < 0.05). Fractional shortening, Ejection fraction were significantly lower in β-TM than TI group (p < 0.001). A statistically significant negative correlation was found between cardiac T2* and each of (IVRT, MPI LV, MPI RV) (p ˂ 0.05).

CONCLUSION: Arrhythmias are more common in the β-TM group. Systolic, diastolic dysfunction and high pulmonary pressure are more prevalent in TM than in TI. Global myocardial performance is more impaired in TM than in TI patients. Iron overload has a deleterious effect on cardiac function.

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References

Sahin C, Basaran O, Altun I, Akin F, Topal Y, Topal H, et al. Assessment of myocardial performance index and aortic elasticity in patients with beta-thalassemia major. J Clin Med Res. 2015;7(10):795-801. https://doi.org/10.14740/jocmr2293w PMid:26346439 DOI: https://doi.org/10.14740/jocmr2293w

Yaman A, Isik P, Yarali N, Karademir S, Cetinkay AS, Bay A, et al. Common complications in beta-thalassemia patients. Int J Hematol Oncol. 2013;3(23):193-9. DOI: https://doi.org/10.4999/uhod.12005

Hershko C. Pathogenesis and management of iron toxicity in thalassemia. Ann N Y Acad Sci. 2010;1202:1-9. https://doi.org/10.1111/j.1749-6632.2010.05544.x PMid:20712765 DOI: https://doi.org/10.1111/j.1749-6632.2010.05544.x

Bornaun H, Dedeoglu R, Oztarhan K, Dedeoglu S, Erfidan E, Gundogdu M, et al. Detection of early right ventricular dysfunction in young patients with thalassemia major using tissue Doppler imaging. Iran J Pediatr. 2016;26(3):e5808. https://doi.org/10.5812/ijp.5808 PMid:27617076 DOI: https://doi.org/10.5812/ijp.5808

Amoozgar H, Zeighami S, Haghpanah S, Karimi M. A comparison of heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major. Hematology. 2016;22(1):25-9. https://doi.org/10.1080/10245332.2016.1226699 PMid:27650671 DOI: https://doi.org/10.1080/10245332.2016.1226699

Bayar N, Arslan S, Erkal Z, Küçükseymen S. Sustained ventricular tachycardia in a patient with thalassemia major. Ann Noninvasive Electrocardiol. 2014;19(2):193-7. https://doi.org/10.1111/anec.12085 PMid:24708271 DOI: https://doi.org/10.1111/anec.12085

Kremastinos DT, Farmakis D, Aessopos A, Hahalis G, Hamodraka E, Tsiapras D, et al. Thalassemia cardiomyopathy history, present considerations, and future perspectives. Circ Heart Fail. 2010;3(3):451-8. https://doi.org/10.1161/CIRCHEARTFAILURE.109.913863 PMid:20484195 DOI: https://doi.org/10.1161/CIRCHEARTFAILURE.109.913863

Russo V, Rago A, Papa AA, Nigro G. Electrocardiographic presentation, cardiac arrhythmias, and their management in β thalassemia major patients. Ann Noninvasive Electrocardiol. 2016;21(4):335-42. https://doi.org/10.1111/anec.12389 PMid:27324981 DOI: https://doi.org/10.1111/anec.12389

Qureshi N, Avasarala K, Foote D, Vichinsky E. Utility of Holter electrocardiogram in iron-overloaded hemoglobinopathies. Ann N Y Acad Sci. 2005;1054:476-80. https://doi.org/10.1196/annals.1345.064 PMid:16339701 DOI: https://doi.org/10.1196/annals.1345.064

Fragasso A, Ciancio A, Mannarella C, Gaudiano C, Scarciolla O, Ottonello C, et al. Myocardial iron overload assessed by magnetic resonance imaging (MRI)T2* in multi-transfused patients with thalassemia and acquired anemias. Eur J Intern. 2011;22(1):62-5. https://doi.org/10.1016/j.ejim.2010.10.005 PMid:21238896 DOI: https://doi.org/10.1016/j.ejim.2010.10.005

Buxton AE, Calkins H, Callans DJ, DiMarco JP, Fisher JD, Greene HL, et al. ACC/AHA/HRS 2006 key data elements and definitions for electrophysiological studies and procedures: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Data Standards (ACC/ AHA/HRS Writing Committee to Develop Data Standards on Electrophysiology). J Am Coll Cardiol. 2006;48(11):2360-96. https://doi.org/10.1016/j.jacc.2006.09.020 PMid:17161282 DOI: https://doi.org/10.1016/j.jacc.2006.09.020

Lai WW, Geva T, Shirali GS, Frommelt PC, Humes RA, Brook MM, et al. Task Force of the Pediatric Council of the American Society of Echocardiography. Guidelines and standards for performance of a pediatric Echocardiogram: A report from the Task Force of the Pediatric Council of the American Society of Echocardiography. J Am Soc Echocardiogr. 2006;19(12):1413-30. https://doi.org/10.1016/j.echo.2006.09.001 PMid:17138024 DOI: https://doi.org/10.1016/j.echo.2006.09.001

Westwood M, Anderson LJ, Firmin DN, Gatehouse PD, Charrier CC, Wonke B, et al. A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging. 2003;18(1):33-9. https://doi.org/10.1002/jmri.10332 PMid:12815637 DOI: https://doi.org/10.1002/jmri.10332

Kirk P, Roughton M, Porter JB, Walker JM, Tanner MA, Patel J, et al. Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major. Circulation. 2009;120(20):1961-8. https://doi.org/10.1161/CIRCULATIONAHA.109.874487 PMid:19801505 DOI: https://doi.org/10.1161/CIRCULATIONAHA.109.874487

Carpenter JP, He T, Kirk P, Roughton M, Anderson LJ, de Noronha SV, et al. On T2* magnetic resonance and cardiac Iron. Circulation. 2011;123(14):1519-28. https://doi.org/10.1161/CIRCULATIONAHA.110.007641 PMid:21444881 DOI: https://doi.org/10.1161/CIRCULATIONAHA.110.007641

Koonrungsesomboon N, Chattipakorn SC, Fucharoen S, Chattipakorn N. Early detection of cardiac involvement in thalassemia: From bench to bedside perspective. World J Cardiol. 2013;5(8):270-9. https://doi.org/10.4330/wjc.v5.i8.270 PMid:24009816 DOI: https://doi.org/10.4330/wjc.v5.i8.270

Mehmood R, Yaqoob U, Sarfaraz A, Zubair U. Complete blood picture with skeletal and visceral changes in patients with thalassemia major. Int J Health Sci (Qassim). 2018;12(4):3-10. PMid:30022897

Wood JC. Cardiac complications in thalassemia major. Hemoglobin. 2009;33 Suppl 1:S81-6. https://doi.org/10.3109/03630260903347526 PMid:20001637 DOI: https://doi.org/10.3109/03630260903347526

Noori NM, Mohamadi M, Keshavarz K, Alavi SM, Mahjoubifard M, Mirmesdagh Y. Comparison of Right and Left Side Heart Functions in Patients with Thalassemia Major, Patients with Thalassemia Intermedia, and Control Group. J The Univ Heart Ctr. 2013;8(1):35-41. PMid:23646046

Barbero U, Longo F, Destefanis P, Gaglioti CM, Pozzi R, Piga A. Worsening of myocardial performance index in beta-thalassemia patients despite permanently normal iron load at MRI: A simple and cheap index reflecting cardiovascular involvement? IJC Metab Endocr. 2016;13:41-4. DOI: https://doi.org/10.1016/j.ijcme.2016.09.003

Abbas AA, Najeb B, Abdulhussein A, Jassim JH, Falih MA, Jubiaer H, et al. Echocardiographic parameters of left ventricle systolic and diastolic function in patients with β-thalassemia major. Iraqi Postgrad Med J. 2012;11(4):562-8.

Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, et al. Thalassemia heart disease: A comparative evaluation of thalassemia major and thalassemia intermedia. Chest. 2005;127(5):1523-30. https://doi.org/10.1378/chest.127.5.1523 PMid:15888823 DOI: https://doi.org/10.1378/chest.127.5.1523

Fraidenburg DR, Machado RF. Pulmonary hypertension associated with thalassemia syndromes. Ann N Y Acad Sci. 2016;1368(1):127-39. https://doi.org/10.1111/nyas.13037 PMid:27008311 DOI: https://doi.org/10.1111/nyas.13037

Panda PK, Sharma Y. Ferritin level: Predictor of thalassemia cardiomyopathy. Heart India. 2018;6(1):18-21. DOI: https://doi.org/10.4103/heartindia.heartindia_8_17

Aessopos A, Berdoukas V. Cardiac function and iron chelation in thalassemia major and intermedia: A review of the underlying pathophysiology and approach to chelation management. Mediterr J Hematol Infect Dis. 2009;1(1):e2009002. https://doi.org/10.4084/MJHID.2009.002 PMid:21415984 DOI: https://doi.org/10.4084/MJHID.2009.002

Barzin M, Kowsarian M, Akhlaghpoor S, Jalalian R, Taremi M. Correlation of cardiac MRI T2* with echocardiography in thalassemia major. Eur Rev Med Pharmacol Sci. 2012;16(2):254-60. PMid:22428478

Djer MM, Anggriawan SL, Gatot D, Amalia P, Sastroasmoro S, Widjaja P. Correlation between T2* cardiovascular magnetic resonance with left ventricular function and mass in adolescent and adult major thalassemia patients with iron overload. Acta Med Indones. 2013;45(4):295-301. PMid:24448334

Leonardi B, Margossian R, Colan SD, Powell AJ. Relationship of magnetic resonance imaging estimation of myocardial iron to left ventricular systolic and diastolic function in thalassemia. JACC Cardiovasc Imaging. 2008;1(5):572-8. https://doi.org/10.1016/j.jcmg.2008.04.005 PMid:19356483 DOI: https://doi.org/10.1016/j.jcmg.2008.04.005

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Published

2022-04-27

How to Cite

1.
Salama K, Khaled HZ, El Dien HMS, Afifi RA-RA-A, Shaheen NMM, El Wahab MAMA. Assessment of Cardiac Functions and Arrhythmia in Children with Beta-Thalassemia Major and Beta-Thalassemia Intermedia. Open Access Maced J Med Sci [Internet]. 2022 Apr. 27 [cited 2024 Dec. 4];10(B):890-5. Available from: https://oamjms.eu/index.php/mjms/article/view/9026