Cardiovascular Status in Regularly Transfused Thalassemia Major Children
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The study was conducted among 100 beta thalassemic children in the age group of 5 to 8 yrs, enrolled at the Thalassemia Unit, Department of Pediatrics, Rajindra Hospital, Patiala, who were diagnosed to have beta thalassemia for atleast 3 years. A detailed history, clinical examination, laboratory workup, electrocardiography and echocardiography were done as per the proforma attached.
Data was analyzed using Chi Square test for significance. The strength of association was assessed using Odd's ratio and its 95% confidence limits. Binary logistic regression analysis was done following a stepwise method, among variable with a value <0.1 in univariate analysis to eliminate confounders.
Among the 100 study subjects 14% had cardiac involvement. Most common age group with cardiac complications was 15 to 18 years (57.14%). Most common cardiac complication associated with beta thalassemia was left ventricular hypertrophy with 50% subjects detected by echocardiography, closely followed by systolic dysfunction. The determinants of cardiac complications among regularly transfused beta thalassemics were subjected to univariate analysis showed tachycardia (p 0.004), lower diastolic blood pressure (p 0.0028), irregular chelation therapy (0.045), higher number of blood pressure (p 0.043) and older age (p 0.000) to have statistically significant association (p<0.05) for cardiac complications. Determinants with p values <0.1 in univariate analysis were further analyzed using multivariate binary logistic stepwise regression. Higher number of blood transfusion (p 0.020), lower pre transfusion hemoglobin (p 0.045) and older age (p 0.003) were noted as the independent determinants of cardiac complications.
The important message to be put out from this study is the presence of factors which can suggest an impending cardiac involvement. Early detection of the same and checking the progression of the disease process to stem the eventual cardiac damage is possible and should be done regularly.
- Kremastions DT, Tsiapras DP, Kostopoulou AG, Hamodraka ES, Chaidaroglou AS, Kapsali ED, NT-proBNP levels and diastolic dysfunction in beta-thalassaemia major patients. Eur J Heart Fail. 2007;531-536.
- Engle MA, Erlandson M, Smith CH. Late cardiac complications of chronic, refractory anemia with hemochromatosis. Circulation. 1964; 30:698-705.
- Kremastinos DT, Toutouzas PK, Vyssoulis GP, Venetis CA, Avgoustakis DG. Iron overload and left ventricular performance in beta thalassemia. Acta Cardiol 1984; 29-40.
- Kilegman, Stanton, St. Geme, Schor, Behrman, Neison Textbook Of Paediatrics, Volume 2, 19th edition, Elsevier Publications, 2011 pages 16741677.
- Ehlers KH, Levin AR, Markenson Al, Marcus JR, Klein AA, Hilgartner MW, et al. Longitudinal study of cardiac function in thalassemia major. Ann N Y Acad Sci. 1980; 344:397-404
- Wood. JC., Enriquez. C, Ghugre. N, Otto-duessel M, Aguilar M,. Neison M D, et al Physiology and Pathophysiology of Iron Cardiomyopathy in Thalassemia Ann N Y Acad Sci. 2005; 1054: 386-395.
- Kremastinos DT, Tsiapras DP. Tsetsos GA, Rentoukas El, Vretou HP, Toutouzas PK. Left ventircular diastolic Doppler characteristics in beta thalassemia major. Circulation. 1993; 1127-1135.
- Rund D, Rachmilewitz E. Beta-thalassemia. N Engl J Med. 2055; 353: 1135-1146.
- Hahalis G, Manolis AS, Apostolopoulos D, Alexopoulos D, Vagenakis AG, Zoumbos NC. Right ventricular cardiomyopathy in betathalassaemiamajor. Eur Heart J. 2002; 23:147-156.
- Ladis V, Chouliaras G, Berdousi H, Kanavakis E, Kattamis C. Longitudinal study of survival and causes of death in patients with thalassemia major in greece. Ann N Y Acad Sci. 2005; 1054:445-450
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