Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study

Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study

Authors

  • Khalfan Al Senaidi Sultan Qaboos University Hospital
  • Surekha Maveda Sultan Qaboos University Hospital
  • Niranjan Joshi Sultan Qaboos University Hospital
  • Hanan Nazir Faculty of Medicine, Alexandria University
  • Mohamed Elshinawy Faculty of Medicine, Alexandria University
  • Shoaib AL Zadjali Sultan Qaboos University Hospital
  • Abdulhakim Al Rawas Sultan Qaboos University Hospital
  • Islam Elghamry Sultan Qaboos University Hospital
  • Yasser Wali a:1:{s:5:"en_US";s:33:"Sultan Qaboos University Hospital";}
  • Doaa Khater Faculty of Medicine, Alexandria University

Keywords:

non-transfusion dependent thalassemia (NTDT), regular blood transfusion, echocardiography.

Abstract

Background and aim of the work:

 Cardiac complications occur in patients with non-transfusion dependent thalassemia (NTDT). The study aimed to evaluate transfusion effect on systolic and diastolic cardiac function in young NTDT patients. 

Methods: 

Study design: Cohort study.

Seventeen regularly-transfused patients with NTDT (12.5±5.3 years; group 1) and 15 none/minimally transfused patients (13.2±4.8 years; group 2) were followed up for 5 years and compared as regards their clinical parameters, echocardiographic and Tissue-Doppler-Imaging.

Results: Group 2 patients had significantly higher peak late-diastolic velocity of the left-ventricular-inflow Doppler (Am). Mitral-valve A-wave duration/pulmonary-veins, A-wave duration-ratio and pulmonary-vein S/D velocities-ratio were larger in group 2 as well (p = < 0.01). The diameters of right and left outflow-tract were larger with a higher cardiac-index in patients of group 2. Systolic-function was similar in the 2 studied groups.

Conclusion:

Diastolic function assessment revealed indicators of an abnormal relaxation of left-ventricle in non-transfused patients, which suggests a diastolic dysfunction. An increase in the diameter of the outflow-tract is likely attributed to high cardiac-output status in non-transfused NTDT patients as they have a higher cardiac index. Early start of regular transfusion for NTDT patients might prevent serious long-term cardiac complications.

Author Biographies

Khalfan Al Senaidi, Sultan Qaboos University Hospital

Child Health Department, Sultan Qaboos University Hospital, Oman;

Surekha Maveda, Sultan Qaboos University Hospital

Child Health Department, Sultan Qaboos University Hospital, Oman

Niranjan Joshi, Sultan Qaboos University Hospital

Child Health Department, Sultan Qaboos University Hospital, Oman

Hanan Nazir , Faculty of Medicine, Alexandria University

Department of Pediatrics

Mohamed Elshinawy, Faculty of Medicine, Alexandria University

Department of Pediatrics

Shoaib AL Zadjali , Sultan Qaboos University Hospital

Department of Hematology

Abdulhakim Al Rawas , Sultan Qaboos University Hospital

Child Health Department

Islam Elghamry , Sultan Qaboos University Hospital

Child Health Department, Sultan Qaboos University Hospital, Oman

Doaa Khater, Faculty of Medicine, Alexandria University

Department of Pediatrics

References

1. Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med. 2005; 353:1135-46.
2. Camaschella C, Cappellini MD. Thalassemia intermedia. Haematologica. 1995; 80:58-68.
3. Tony S, Daar S, Elshinawy M, Al-Zadjaly S, Al-Khabori M, Wali Y. T2* MRI in regularly transfused children with thalassemia intermedia: serum ferritin does not reflect liver iron stores. Pediatr Hematol Oncol. 2012; 29:579-84.
4. Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood. 2001; 97:3411-16.
5. Daar S, Gravell D, Hussein HM, Pathare AV, Wali Y, Krishnamoorthy R. Haematological and clinical features of beta-thalassemia associated with Hb Dhofar. Eur J Haematol. 2008; 80:67-70.
6. Taher AT, Musallam KM, Karimi M, et al. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2010; 115:1886-92.
7. Karimi M, Musallam KM, Cappellini MD, et al. Risk factors for pulmonary hypertension in patients with beta thalassemia intermedia. Eur J Intern Med. 2011; 22:607-10.
8. Aessopos A, Kati M, Farmakis D. Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica. 2007; 92:658-65.
9. Abdurrahman L, Hoit BD, Banerjee A, Khoury PR, Meyer RA. Pulmonary venous flow Doppler velocities in children. J Am Soc Echocardiogr. 1998; 11:132-137.
10. Eidem BW, McMahon CJ, Cohen RR, et al. Impact of cardiac growth on Doppler tissue imaging velocities: a study in healthy children. J Am Soc Echocardiogr. 2004; 17:212-21.
11. O'Leary PW, Durongpisitkul K, Cordes TM, et al. Diastolic ventricular function in children: a Doppler echocardiographic study establishing normal values and predictors of increased ventricular end-diastolic pressure. Mayo Clin Proc. 1998; 73:616-28.
12. Alkindi SS, Alzadjali S, Daar S, et al. A stepwise alpha-thalassemia screening strategy in high-prevalence areas. Eur J Haematol. 2013; 91:164-9.
13. dos Santos CO, Zhou S, Secolin R, et al. Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function. Am J Hematol. 2008; 83:103–8.
14. Lopez L, Colan SD, Frommelt PC, et al. Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr. 2010; 23:465-95.
15. Lewis JF, Kuo LC, Nelson JG, Limacher MC, Quinones MA. Pulsed Doppler echocardiographic determination of stroke volume and cardiac output: clinical validation of two new methods using the apical window. Circulation 1984; 70;425-31.
16. Cantinotti M, Giordano R, Scalese M, et al. Nomograms for mitral inflow doppler and tissue doppler velocities in caucasian children. J Cardiol. 2016; 68:288–99.
17. Taher AT, Musallam KM, Cappellini MD, Weatherall DJ. Optimal management of beta thalassemia intermedia. Br J Haematol. 2011; 152:512-23.
18. Aessopos A, Kati M, Meletis J. Thalassemia intermedia today: should patients regularly receive transfusions? Transfusion. 2007; 47:792-800.
19. Olivieri NF. The beta-thalassemias. N Engl J Med. 1999; 341:99-109.
20. Aessopos A, Farmakis D, Deftereos S, et al. Thalassemia heart disease: a comparative evaluation of thalassemia major and thalassemia intermedia. Chest. 2005; 127:1523-30.
21. Amoozgar H, Farhani N, Karimi M. Early echocardiographic findings in beta-thalassemia intermedia patients using standard and tissue Doppler methods. Pediatr Cardiol. 2011; 32:154-9.
22. Isma'eel H, Chafic AH, El Rassi F, et al. Relation between iron-overload indices, cardiac echo-Doppler, and biochemical markers in thalassemia intermedia. Am J Cardiol. 2008; 102:363-7.
23.Tony S, Daar S, Zachariah M, Wali Y. Prepubertal Hypertransfusion in Thalassemia Intermedia: Sustained Positive Effects on Growth, Splenic Function and Endocrine Parameters. Oman Med J. 2012; 27:125-8.
24.Vaccari M, Crepaz R, Fortini M, et al. Left ventricular remodeling, systolic function, and diastolic function in young adults with beta-thalassemia intermedia: a Doppler echocardiography study. Chest. 2002; 121:506-12.

Downloads

Published

11-05-2022

Issue

Section

ORIGINAL ARTICLES

How to Cite

1.
Al Senaidi K, Maveda S, Joshi N, Nazir H, Elshinawy M, AL Zadjali S, et al. Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study . Acta Biomed [Internet]. 2022 May 11 [cited 2024 Jul. 17];93(2):e2022022. Available from: https://mattioli1885journals.com/index.php/actabiomedica/article/view/10736