Examination of heart and respiratory functions in patients with beta thalassemia major

ABSTRACT Aim: Beta thalassemia is a genetic disease, which is characterized by chronic hemolytic anemia and transfusion dependence. Chronic transfusions lead to iron overload in many organs and causes organ dysfunction primary heart failure. We aimed to determine the respiratory and cardiac functions of multi-transfused thalassemia patients and their relationship between them. Material and Method: Thirty-five thalassemia major patients were enrolled to the study. Respiratory function test, M-mode, PW Doppler and tissue doppler echocardiography were performed. The obtained data were compared with the groups according to sex, mean ferritin value, number of total transfusions and splenectomy status. Pulmonary function test results and echocardiography results were also compared. Statistical analysis was performed with the IBM SPSS 22.0 program. Results: Thirty-five patients were included to the study, 13 female, 22 male. The mean age of the patients was 10,8 (range 5-18). Congestive heart failure was not detected in any of our patients. The 34.3% of patients had splenectomy. Respiratory function tests; revealed normal in 12 patients (37.5%), isolated restrictive failure in 3 (9.4%), restrictive and distal airway obstruction together in 17 patients (53.1%). Respiratory dysfunction was detected in 20 (62.5%) of our patients. There were no significant differences between the males and females in terms of pulmonary function test parameters and results (p>0.05). There were no statistically significant difference between the results of splenectomized patients, total transfusion number, mean ferritin values, and the pulmonary function test results (p>0.05). Left ventricular measurements were found to be higher in splenectomized patients, mean ferritin value > 1500 ng / ml and transfusion number > 200 compared to the other groups (p<0.05). There were no statistically significant difference between the results of PW Doppler, Doppler echocardiography parameters ,splenectomy status, mean ferritin value, total transfusion counts and pulmonary function test results (p>0.05). Conclusions: In our patients, the most common pulmonary dysfunction was restrictive and distal airway obstruction together. Left ventricular measurements of our patients were higher in splenectomized, multi-transfused patients and patients with high ferritin levels. When we compared the data of respiratory function test and doppler echocardiography in our study, no significant difference was found between the groups. Keywords: Thalassemia major, Doppler echocardiography, Respiratory functions,Children