The Role of Heart Rate Variability and Fragmented QRS for Determination of Subclinical Cardiac Involvement in Beta-Thalassemia Major.

BACKGROUND: Iron accumulation leads to increased susceptibility to cardiovascular diseases in thalassemia major (TM) patients. Depressed heart rate variability (HRV) and development of arrhythmia are among the manifestations of subclinical cardiac involvement in TM cases. Determination of subclinical cardiac involvement is essential for preventive measures. Thus, we aimed to evaluate the best method for identification of subclinical cardiac dysfunction in TM cases. MATERIALS AND METHODS: In this prospective study, 45 TM and 45 non-TM cases, who were referred for cardiac evaluation, were enrolled. Exclusion criteria included non-sinus rhythm and overt cardiac disease. TM cases underwent cardiac MRI, electrocardiography (ECG), and Holter monitoring. TM cases were divided into two groups of normal versus iron overload with a cardiac T2* of more or less than 20 ms, respectively. The non-TM cases underwent only ECG and Holter monitoring. RESULTS: We observed no significant difference regarding HRV between normal versus iron overload TM and non-TM cases. Higher rates of premature atrial complex, low limb voltage, low atrial rhythm, as well as minimum and average HR with lower QRS duration and PR interval were detected in TM versus non-TM cases (p value <0.05). CONCLUSIONS: We observed a higher prevalence of low limb voltage and low atrial rhythm in TM cases versus non-TM cases. Indeed, the role of fragmented QRS (fQRS) for subclinical detection of cardiac disease in TM cases is still so controversial and needs more evaluation. Application of HRV and fQRS in this regard may need to be performed at the right time point after initiation of blood transfusion, but this needs to be determined.

Efficacy of Deferasirox (Exjade®) in Modulation of Iron Overload in Patients with ß-Thalassemia Intermedia.

Because of insufficient erythropoiesis, peripheral hemolysis and increased gastrointestinal iron absorption, iron overload is still a matter of debate in ß-thalassemia intermedia (ß-TI) patients, which can be overcome using iron chelators. However, data on use of iron chelators in ß-TI patients is highly restricted. The aim of this study was to evaluate the efficacy of oral administration of deferasirox (Exjade(®) or DFX) by assessment of serum ferritin levels in ß-TI patients. In this quasi-experimental study, 50 ß-TI patients with serum ferritin levels >1000 ng/mL were selected and received oral DFX for 12 consecutive months. Iron overload was measured by checking serum ferritin levels every 2 months and the results were compared with the baseline level. The mean serum ferritin was decreased during 1 year of chelation therapy without any toxic effect. Although the difference between baseline ferritin and ferritin levels at the end of second month was not remarkable (p = 0.88), a significant reduction in serum ferritin was observed after 4 (p = 0.01), 6 (p = 0.001), 8 (p < 0.001), 10 (p < 0.001) and 12 months (p < 0.001) of chelation therapy compared to its baseline levels. There was no correlation between baseline ferritin levels and age (p = 0.574). In addition, no statistically significant difference was observed about change in serum ferritin levels after 6 and also 12 months of therapy between patients who had undergone splenectomy and those who did not (p = 0.796 and 0.859, respectively). Iron chelation therapy with DFX is safe and effective in reducing serum ferritin levels in ß-TI patients who suffer from side effects of iron overload.

Anxiety and depression affects life and sleep quality in adults with beta-thalassemia.

PURPOSE: The aim of this study was to investigate the association of anxiety and depression symptoms with health related quality of life (HRQoL) and sleep quality in patients with beta-thalassemia. METHODS: In a cross-sectional study between 2006 and 2007, 292 thalassemic patients were assessed for symptoms of anxiety and depression (Hospital Anxiety Depression Scale; HADS), HRQoL (Short Form-36, SF-36) and quality of sleep (Pittsburgh Sleep Quality Index; PSQI). Linear regression models were used to determine possible predictive value of high anxiety and depressive symptoms on HRQoL and sleep quality, separately. RESULTS: Mental and physical quality of life scores were predicted by symptoms of depression and somatic comorbidities. Total sleep quality was predicted by anxiety symptoms and somatic comorbidities. CONCLUSIONS: Screening for anxiety and depression in patients with thalassemia is essential. Further studies should test if appropriate treatment of these conditions may improve patients HRQoL and sleep quality or not.