An Echocardiographic Evaluation of Left Ventricular Function in Patients with Thalassemia Major

BACKGROUND: Cardiac disease caused by transfusional iron overload remains the principal cause of death in patients with β- thalassemia major, despite improvements in iron chelation therapy during the past 25 years. For this reason, regular evaluation of cardiac function is recommended for all patients with thalassemia major and is now an integral part of their management. Cardiac monitoring should, therefore, ideally identify patients at highest risk for cardiac decompensation before heart failure develops. Sequential and reproducible quantification of ventricular function in principle can identify early changes in the left ventricular ejection fraction from baseline for each patient and could be used as a rationale for identifying patients at high risk. METHODS: The study was conducted in the Department of Pediatric Cardiology, The Children's Hospital and Institute of Child Health Lahore, Pakistan from 1st April 2006 to 30^(th) October 2010. The study comprised of 50 consecutive confirmed cases of β thalassemia major and 30 controls with normal hemoglobin and electrophoresis pattern. β thalassemia major patients were diagnosed on the basis of hemoglobin electrophoresis. 2-D, M-mode and Doppler echocardiography was performed in all study cases and in control participants. Statistical comparison of study cases and controls was conducted by using unpaired t-test. RESULTS: The age of the patients ranged from 1 year to 25 years with mean age of 9.65 years. There were 34 (68%) males and 16 (32%) females. None of the study cases was on regular chelation while 31 (62%) patients were on irregular chelation therapy with single dose of intravenous desferrioxamine only at the time of blood transfusion. Nineteen (38%) patients had LV dysfunction, of which isolated systolic dysfunction was in 2 (4%), isolated diastolic dysfunction was in 15 (30%) and global dysfunction was in 2 (4%) patients. Left ventricular dimensions, stroke volume and E/A ratio were considerably affected in the study group. CONCLUSION: A significant percentage of thalassemia patients have left ventricular dysfunction. This is mainly due to chronic anemia, iron overload and poor compliance with chelation therapy. Regular assessment of cardiac function may help to improve the quality of life of these patients and may reduce the morbidity and mortality to a great extent.

並列關鍵字

Beta thalassemia major ； Echocardiography ； Left ventricular dysfunction

國際替代計量

全文下載

主題瀏覽