Prevalence of pulmonary artery hypertension (pah) in patients with thalassemia

Background and objective Lack of regular blood transfusions in patients with intermediate thalassemia leads to a higher probability of pulmonary arterial hypertension (PAH) in comparison with other patients. PAH remain a concern in thalassemia patients. The aim of this study was to find the prevalence of PAH in patients with thalassemia. Methods In this Cross-Sectional study, sixty three matched patients were consecutively admitted to hospital and divided to two groups (29 intermediate and 34 major thalassemia patients). Echocardiography, Complete Blood Count (CBC) and ferritin analysis were done for both groups. The mean pulmonary artery systolic pressure (PASP) values ≥25 mmHg defined as PAH. Patients with TI were treated by Hydroxyurea and didn't need regular monthly blood transfusions for maintaining their hemoglobin levels above 9.5. Data collected for each group patients and then analyzed by Statistical methods in SPSS.19. Results The prevalence of PAH in patients with TI with 12(41.4%) was significantly 2.8 time higher than patients with TM with 5(14.7%).(p=0.018) Variables such as sex, marital status, age, nucleated red blood cells(NRBC),heart failure, ECG change rate were similar between two groups. Conclussion: PAH in patients with thalassemia intermediate which treated with Hydroxyurea were significantly more than patients with thalassemia major which receiving regular blood transfusions. Therefore, it was recommended that before appearance the symptoms of pulmonary hypertension in the patients with thalassemia intermediate, especially males, regular blood transfusions to be started in these groups