Evaluation of heart disease in patients with beta-thalessemia major referred to Shahid Madanni Hospital in Khorram Abad in 2003

Abstract

Background: Patients with beta-thalessemia major present with severe anemia and need continous transfusion therapy. An important complication of beta-thalessemia major is iron deposition in cardiac tissue, resulting in fibrosis and dysfunction. Cardiac involvement is the major cause of death in beta-thalessemia major. The purpose of this study was to assess cardiac disease patients with beta-thalessemia major. Materials and methods: A cross-sectional study was done on 100 patients affected by beta-thalessemia major who referred to Shahid Madani Hospital in Khorram Abad in 2003. Factors studied included age, sex, serum ferretin, patient's age at the time of the initial blood transfusion, patient's age at the time of Desferal (deferoxamine mesylate) use, blood group, and hemoglobin level. All patients were examined and had echocardiographies performed by a cardiologist. Data was collected by questionnaire, and an analysis was made using the SPSS program. Results: 33% of the patients had cardiac disease. Six patients had restrictive type systolic dysfunction of the left ventrical and diastolic left ventricular dysfunction with 50-55% ejection fraction. Two patients had four-chamber enlargement and high output cardiomyopathy failure with 45-55% ejection fraction. Two patients had severe pulmonary hypertension, aortic and tricuspid valve insuficiency, right and left ventricular dysfunction, pericardial effusion with 45-55% ejection fraction. The remaining patients had left ventricular enlargement alone or with right ventricular enlaregement with 60% ejection fraction. 54.5% of the cardiac patients were male and 45.5% were female. In 23% of the patients, serum ferretin was 1501-2000 nanograms per ml. Most of the patients were 11-20 years old, and the age of initial blood transfusion in 20% of the patients was 1-2 years of age. The age at the time of Desferaluse in 25% of the patients was after five years of age. Most of the patients had type O blood. Two patients died from heart failure. Conclusions: Based on the X2 test, there was a postive correlation between age, age of Desferal use, and serum ferretin with cardiac disease (P<0.05). However, there was no correlation between age and blood group with cardiac disease. It is suggested that patients with beta-thalessemia major have blood transfusions and use Deseral regularly. Also, it is necessary to periodically evaluate the heart function of these patients.