Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study
Ahmed, A., Ali, S., Madkoor, W. (2018). Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study. EKB Journal Management System, 22(1), 439-444. doi: 10.21608/smj.2018.42525
Alzahraa Alsayed Ahmed; Safaa Husein Ali; Walaa Mostafa Madkoor. "Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study". EKB Journal Management System, 22, 1, 2018, 439-444. doi: 10.21608/smj.2018.42525
Ahmed, A., Ali, S., Madkoor, W. (2018). 'Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study', EKB Journal Management System, 22(1), pp. 439-444. doi: 10.21608/smj.2018.42525
Ahmed, A., Ali, S., Madkoor, W. Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study. EKB Journal Management System, 2018; 22(1): 439-444. doi: 10.21608/smj.2018.42525

Evaluation of cardiac function in patients with thalassemia intermedia: A prospective hospital based study

Sohag Medical Journal
Article 60, Volume 22, Issue 1, January 2018, Page 439-444  XML PDF (71.74 K)
Document Type: Original Article
DOI: 10.21608/smj.2018.42525
View on SCiNiTO View on SCiNiTO
Authors
Alzahraa Alsayed Ahmed1; Safaa Husein Ali2; Walaa Mostafa Madkoor3
1Department of Pediatric haematology Unit, Pediatric Department,Faculty of Medicine, Sohag University, Sohag, Egypt.
2Department of Pediatric , Faculty of Medicine,Sohag University, Sohag, Egypt.
3Department of Pediatric ,Faculty of Medicine, Sohag University, Sohag Egypt.
Abstract
Background:B-Thalassemia is an inherited hemoglobin disorder caused by impaired synthesis of the b-globin chain and resulting in chronic hemolytic anemia.In the absence of regular treatment of thalassemia intermedia patients the diastolic performance of the left ventricle is maintained stable, while the pressure of pulmonary arteries continues to rise which is the main cause of death in these patients. 2D, M mode echocardiographyand tissueDopplerare method for evaluating the cardiac function in patients with thalassemia intermedia.
Objective: Our main objective in this study is to evaluate the cardiac function in patients with thalassemia intermedia by 2D, M mode echocardiography and Doppler methods.
Methods: This case-control study was conducted on 46 thalassemic patients (mean age: 9.7 ± 4.6years) and 46 healthy individuals (mean age: 10.3 ± 4.1 years) as a control group from October 2016 toSeptember 2017 in the Pediatric haematology Clinics and inpatients of Pediatric Department.There was no sign of cardiac involvement by physical examination, chest x-ray and ECG in patients. Echocardiographic parameters were measured in groups, and finally data was analyzed by SPSS software.
Results:The mean of left ventricular myocardial performance index (LMPI) ((P- value<0.001) and left ventricular mass index (LVMI) ((P- value<0.001) have statistically significant differencebetween two groups. Mean of interventricular septal dimension in diastole (IVSD), left ventricular posterior wall thickness in diastole (LVPWD) and interventricular septal dimension in systole (IVSS) were also statistically significant with a P-value of, <0.001, 0.042, 0.05).
Conclusion: the systolic and diastolic performance of thalassemia intermedia patients is affected earlier in comparison with control group in some parameters including LVMPI, EF, FS, IVSD, IVSS, LVPWDD and RVMPI. For more evaluation, more quantitative and comprehensive research suggested by evaluation of specific effective factors to psychosocial health of these patients.
Study Limitation
* Age group: we conduct only the pediatric age group thalassemia intermedia, and as we know the most of complication appear in older age
* Number of patient: we conduct only46 patient in our study.
*Lack of material potential: availability of other facilities like T* and MRI as echocardiography is not able to replace them even echocardiographic abnormalities are found.
Keywords
thalassemia intermedia in children; Complications of TI; cardiac function; echocardiography and Dopplerstudies
Supplementary Files
References
  1.  Aessopos A , Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, Karagiorga M. Thalassemia Heart Disease: A Comparative Evaluation of Thalassemia Major and Thalassemia Intermedia. Chest. 2005; 127(5):1523-1530.
  2. 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. Mediterranean Journal of Hematology and Infectious Diseases.2009; 49 (2):137-143.
  3. Aessopos A, Tsironi M, Andreopoulos A, Farmakis D. Heart disease in thalassemia intermedia. Hemoglobin. 2009; 33(1):170-6.
  4. Bosi G, Crepaz R, Gamberini MR, Fortini M, Scarcias, Bonsante E. Left Ventricular remodeling and Systolic and diastolic Function in young adult with beta thalassemia Major: A Doppler echocardiographic assessment and correlation with hematologic data. Heart. 2003; 89(7):262-6.
  5. Challenor VF, Conway N, Monro JL. The surgical treatment of restrictive cardiomyopathy in pseudoxanthoma elasticum. Br Heart J. 2004; 59:266-269.
  6. Ehlers KH, Levin AR, Markenson AL. Longitudinal study of cardiac function in thalassemia major. Ann N Y Acad Sci. 2006; 344:397-404.
  7. Isner JM, Carter BL, Bankoff MS, Konstam MA, Salem DN. Computed tomography in the diagnosis of pericardial heart disease. Ann Intern Med.1982; 97:473-479
  8. Kanavakis E, Traeger-Synodinos J, Tzetis M, Metaxotou-Mavromati A, Ladis V, Kattamis C.Molecular characterization of homozygous (high hba2) beta-thalassemia intermedia in Greece. Pediatric Hematol Onco. 2007;l:135-158.
  9. Mahdi, Safa A, Laith S, Faraj, Hasanein H, Ghali. Significance of Red Blood Cell Indices in Beta-Thalassaemia Trait. Mustansiriya Medical Journal.215; 14:27-30.
  10. Marshall A. Lichtman Ernest Beutler, Thomas J.Williams. Hematology. In: Kaushansky SK, Josef T.7th ed. 2006: 633-6.
  11. Modell B, Berdoukas V. The Clinical Approach to Thalassemia. New York, NY: Grune & Stratton.1984.3:115-130.
  12. Nouri NM, Naderi  M, Emamdadi A, Fadaie M, Rajaie S. Comparison of Cardiac Function in Young Patients with Thalassemia Intermedia and Healthy Individuals Using Echocardiography Method. Iranian journal of blood and cancer. 2012; 4(4):157-161.
  13. Nouri NM, Naderi M , Rajaie S, Dorgalaleh A , Tabibian Sh. Evaluation of Cardiac Function in Patients with Thalassemia Intermedia. Iranian Journal of Pediatric Hematology Oncology. 2013; l3 (1):193-199.
  14. Ocal B, Oguz D, Karademir S, Birgen D, Yüksek N, Ertem U. Myocardial performance index combining systolic and diastolic myocardial performance in doxorubicin-treated patients and its correlation to conventional echo/Doppler indices. Pediatr Cardiol. 2002; 23(5):522-7.
  15. Shawky RM, Kamal TM. Thalassemia intermedia: An overview. Egyptian Journal of Medical Human Genetics. 2012; 13: 245–255.
  16. Vaccari M, Crepaz R, Fortini M, Gamberini MR, Scarcia S, Pitscheider W,. Left ventricular remodeling, systolic function and diastolic function in young adults with beta thalassemia intermedia: a Doppler echocardiography study. Chest 2002; 121(2): 506-12.
  17. Zurlo MG, DE Stefano P, Borgna-Pignatti. Survival and causes of death in thalassemia major. Lancet. 1999; 2:27-30.
Statistics
Article View: 189
PDF Download: 159