Evaluation of systolic function in patients with beta Thalassemia major in Booali Sina Hospital, Sari, 1999

Abstract

Objective: Congestive heart failure and other cardiac complications are the most important causes of death in patients with beta Thalassemia major. Iron overloads and chronic anemia are the major causes of cardiac involvement. The purpose of this study was to evaluate the ability of echocardiographic systolic function for diagnosis of cardiac involvement in patients with Thalassemia major. Methods: This descriptive study was done on patients with Thalassemia major (Over 12 years old) who referred to Booali Sina hospital in Sari. They were evaluated according to their ages, sex, age at beginning of transfusion of deferoxamine, the amount of deferoxamine, serum ferritin level, clinical manifestations, electrocardiogram, chest radiography and echocardiographic systolic function. Data was statistically analyzed by T-test. Findings: 55% of patients were male and the average age was 18±6 years. 22% of patients had systolic dysfunction. The difference of ejection fraction and fractional shortening between patients with and without cardiac involvement was significant (p<0.000). The age at beginning of deferoxamine and ferritin level in patients with cardiac complication was greater. The evaluation of cardiac function for determining cardiac involvement in patients with Thalassemia major with respect to clinical manifestations had a specificity of 76%, sensitivity of 41% and efficacy of 68%. Conclusion: When the patient is clinically symptomatic, systolic function will be impaired. Evaluation of echocardiographic systolic function is not enough for determining cardiac involvement in asymptomatic patients.

Keywords

• thalassemia major
• echocardiography
• systolic function