EVIDENCE OF ABNORMAL LEFT VENTRICULAR FUNCTION IN PATIENTS WITH THALASSAEMIA MAJOR: AN ECHOCARDIOGRAPHY BASED STUDY
AbstractBackground: Thalassaemia represent one of the most common single gene disorder causing a majorpublic health problem in Pakistan. Nearly 100,000 people are born worldwide with this severe blooddisorder every year. Over the last 3 decades, the development of regular transfusion therapy and ironchelation has dramatically improved the quality of life and transformed thalassaemia from a rapidlyfatal disease to a chronic disease compatible with prolonged survival. Objective of this observationalcross sectional study was to determine the effects of chronic anaemia and transfusional iron overload onthe left ventricular function using Doppler echocardiography. Methods: This study was conducted inthe Department of Paediatric Cardiology, The Children’s Hospital & Institute of Child Health, Lahore,Pakistan from 1st April 2006 to 30th September 2007. The study comprised of 50 consecutive cases ofbeta-Thalassaemia major and 30 controls with normal haemoglobin and electrophoresis pattern. β-Thalassaemia major patients were diagnosed on the basis of haemoglobin electrophoresis. Patients withany congenital or acquired heart disease, concurrent infective disorder and with history of cardiacsurgery were excluded from the study. 2-D, M-mode and Doppler echocardiography was performed inall the study cases and controls. Statistical comparison of study cases and controls was conducted byusing unpaired t-test. Results: The age of the patients ranged from 2 years to 25 years with mean age of9.65 years. Males were 34 (68%) and females were 16 (32%). None of the study cases was on regularchelation programme while 31 (62%) patients were on irregular chelation with single dose ofintravenous desferrioxamine only at the time of blood transfusion. 19 (38%) of the patients had LVdysfunction in the form of isolated systolic dysfunction in 2 (4%), isolated diastolic dysfunction in 15(30%) while global dysfunction in 2 (4%) of the patients. Left ventricular dimensions, stroke volumeand E/A ratio were found considerably high in the study group. Conclusion: A very high percentage ofThalassaemia patients have cardiac involvement as LV dysfunction. This is mainly due to chronicanaemia, poor compliance with chelation therapy and non-availability of proper cardiac monitoring.Regular assessment of cardiac function may help a lot to improve the quality of life of these patientsand may reduce the morbidity and mortality to a great extent.Keywords: Beta Thalassaemia major, Echocardiography, Left ventricular dysfunction
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