Left Ventricular Dysfunction in chronically transfused Patients with Thalassemia Major

Background: thalassemia is one of the most common single gene disorders effecting the population of pakistan. it has been found to be one of major health problem in our country leading to morbidity as well as mortality. one of the major causes for both morbidity as well as mortality is due to the effect of the deposition of iron within the myocardium,which predominantly leads to heart failure. this can be either systolic or diastolic heart failure and may involve either the left or the right ventricle. echocardiography helps to recognize ventricular dysfunction. aim: to determine the frequency of left ventricular dysfunction in chronically transfused children with thalassemia major having iron overload. methods: the descriptive cross-sectional survey was conducted at children hospital & institute of child health,lahore from 10-12-2012 to 10-06-2013. two hundred and sixty diagnosed cases of thalassemia major,aged up to 18years were included in the study by purposive non-probability sampling. children with history of acquired or congenital heart disease,concomitant infective process (aided by chest x-ray and echocardiography) and with previous history of cardiac surgery were excluded. transfusional iron overload was defined as serum ferritin >1000ng/dl and left ventricular dysfunction in terms of ejection fraction (ef) (<30%). results: two hundred and sixty patients with mean age 9.3 years±3.93 and mean weight 27.5 kg±11.1 were studied. mean serum ferritin came out 3067±1276 ng/dl while mean ef was 44.7±12.1%.10% patients were <30% ef while 90% had ef >30%. serum ferritin was negatively correlated with ef in thalassemia patients. conclusion: early left ventricular dysfunction is common in cases of thalassemia major with iron overload in chronically transfused children. there is need of timely and periodic screening of left ventricular dysfunction by echocardiography.