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Background: Heart disease is the leading cause of mortality and morbidity in patients with beta-thalassemia. Vitamin D and calcium deficiency are common in these patients. We studied and compared vitamin D and calcium levels and other parameters with echocardiographic findings in patients with beta-thalassemia. Methods: A cross-sectional study on patients with transfusion-dependent thalassemia was conducted. One hundred sixty-nine patients with transfusion-dependent thalassemia were enrolled. Ejection fraction, valvular insufficiency, and aortic diameter were determined. The aortic diameter of patients was measured using the Mindray DC60 echo model. All of these patients were tested for levels of vitamin D, calcium (Ca), parathyroid hormone (PTH), alkaline phosphatase (ALP), platelet (PLT), and ferritin. Finally, the effect of these factors on aortic root (AR), aortic valve area (AVA), and ejection fraction (EF) was evaluated. One-way ANOVA was used to compare quantitative variables, and a chi-square test with a 95% confidence level was used to estimate relationships and compare ratios in groups. Results: A statistically significant relationship existed between vitamin D deficiency and serum Ca (P value=0.009). Our results showed that EF also increased with the increase in vitamin D levels, and the probability that the AR would have a normal size increased. Furthermore, abnormal PTH and PLT levels caused a decrease in EF. Conclusion: This study showed an association between vitamin D deficiency and cardiac function in patients with transfusiondependent thalassemia. Vitamin D can be considered a supplement for thalassemia patients [ABSTRACT FROM AUTHOR]

Background: Cardiac iron overload causes severe cardiac complications and is a leading cause of death in beta-thalassemia major (B-TM) patients. T2-weighted cardiovascular magnetic resonance (T2*CMR) can detect preclinical cardiac iron overload. We evaluated the ability of 12-lead electrocardiographic atrial and ventricular depolarization and repolarization parameters to predict cardiac iron loading in TM. Methods: This cross-sectional study was conducted on patients with beta-thalassemia major; all participants underwent a standard 12-lead electrocardiogram during a single study visit, and the depolarization and repolarization parameters of ECG were measured and compared with the cardiac iron level detected by T2*CMR, with a detectable cardiac iron cutoff of T2* less than 20 ms. Results: A total of 26 patients (mean age of 26.19 years, 34.62% male) were included. Mean T2*CMR values were 21.53 ms (46.15%<20 ms, 53.85%≥20 ms). Among ECG parameters, only Pwd, QTc, and QTcd (P values 0.026, 0.030, and 0.006, respectively) were significantly prolonged in patients with T2*<20 ms compared to patients with T2*≥20 ms. There was a statistically negative correlation between T2*CMR, Pwd, and QTcd (P values 0.028 and 0.021, respectively). Moreover, no correlation was found between Tp-e, Tp-e d, JTc, JTcd, Tp-e/QT, Tp-e/JT, Tp-e/JTc, and T2* values. Conclusion: Pwd and QTcd can be used as alternatives to T2*CMR to predict cardiac iron load levels in patients with beta-thalassemia major [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) T2* is the gold standard for detecting iron deposition in cardiac tissue, but the technique has limitations and cannot be fully performed in paediatric thalassemia patients. The aim of this study was to analyse clinical data to identify other predictors of cardiac iron deposition. A retrospective analysis was performed on 370 children with β-TM. According to the cardiac MRI results, patients were allocated to a cardiac deposition group and noncardiac deposition group. Multivariate analysis revealed that genotype and corrected QT interval were associated with cardiac iron deposition, indicating that the-β0/β0 genotype conferred greater susceptibility to cardiac iron deposition. Receiver operating characteristic curve (ROC) analysis was performed, and the area under the curve (AUC) of genotype was 0.651. The AUC for the corrected QT interval was 0.711, at a cut-off value of 418.5 ms. ROC analysis of the combined genotype and corrected QT interval showed an AUC of 0.762 with 81.3% sensitivity and 64.7% specificity. Compared to patients with the β+/β+ and β0β+ genotypes, β0β0 children with β-TM were more likely to have cardiac iron deposition. Conclusion: The genotype and QTc interval can be used to predict cardiac iron deposition in children with β-TM who are unable to undergo MRI T2 testing. [ABSTRACT FROM AUTHOR]

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Beta thalassemia major (TM) is a common hereditary disease in Indonesia. Iron overload due to regular transfusion may induce myocardial iron deposition leading to electrophysiological dysfunction and functional disorders of the heart. Ventricular arrhythmia is one of the most common causes of sudden cardiac death in thalassemia patients. This cross-sectional study of 62 TM patients aged 10–32 years in Cipto Mangunkusumo General Hospital was done to assess their electrophysiological properties and heart rate variability, including 24- hour Holter monitoring, signal averaged electrocardiogram (SAECG) for detection of ventricular late potential (VLP), and determination of heart rate variability (HRV). We also assessed their 12-lead ECG parameters, such as P wave, QRS complex, QT/ QTc interval, QRS dispersion, and QT/ QTc dispersion. Iron overload was defined by T2-star magnetic resonance (MR-T2*) values of less than 20 ms or ferritin level greater than 2500 ng/mL. Subjects were grouped accordingly. There were significant differences of QTc dispersion (p = 0.026) and deceleration capacity (p = 0.007) between MR-T2* groups. Multivariate analysis showed an inverse correlation between QTc dispersion and MR-T2* values. There was a proportional correlation between heart rate deceleration capacity in the low MR-T2* group (p = 0.058) and the high ferritin group (p = 0.007). No VLPs were detectable in any patients. In conclusion, prolonged QTc dispersion and decreased heart rate deceleration capacity were significantly correlated with greater odds of iron overload among patients with Thalassemia major. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension is one of the most common cardiac complications among patients with β-thalassemia major (β-TM). The aim of this study is to investigate the correlation of pulmonary artery pressure (PAP), serum ferritin level, and cardiac T2* MRI in patients with β-TM. Method: This cross-sectional study was performed on 50 patients older than 7 years old. Echocardiography, electrocardiography, and cardiac T2* MRI were performed on all patients and their serum ferritin levels were measured. Based on the echocardiographic the patients were divided into two groups of PAP>30 and <30 mmHg. Results: 40% had PAP higher than 30 mmHg, 32% had abnormal T2* MRI, and 36% had serum ferritin levels higher than 1500ng/dl. there were significant negative correlations between CMRI *T2 and PAP (-0.36) and the P-pulmonary (-0.29). the serum ferritin level was positively correlated with PAP (0.44) and the P pulmonary in ECG (0.30). in the patients with PAP>30, the means of age, serum ferritin level, and P-pulmonary were significantly higher than patients with PAP<30 (P=0.001). In the patients with PAP>30mmHg, CMRI *T2 had higher accuracy (80%), sensitivity (65%), and specificity (90%) compared to the measurement of serum ferritin levels. Conclusion: The PAP was positively correlated with serum ferritin levels and negatively with T2MRI. The accuracy of 80% for T2MRI as a method to diagnose or even anticipate PH among patients with TM suggested that this method is a useful and accurate method of PH diagnosis and can be used as an alternative among clinicians. [ABSTRACT FROM AUTHOR]

Copyright of Álabe is the property of Alabe and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The prolongation of cardiac repolarization ( QT interval) has been investigated in studies of patients with secondary iron overload. However, no previous population-based study examining the effect of increased iron stores on QT interval prolongation has previously been undertaken. We tested the hypothesis that increased iron stores and haemochromatosis genotype (genetically increased iron stores) are associated with prolongation of the QT interval. We included 20 261 individuals from the Danish General Suburban Population Study and examined differences in QT interval according to ferritin concentration, transferrin saturation, iron concentration, transferrin concentration and haemochromatosis genotype (C282Y/C282Y). Furthermore, we performed a meta-analysis of case-control studies on thalassaemia major patients and QT interval. Age- and C-reactive protein-adjusted mean corrected QT ( QTc) intervals for ferritin concentration ≥99% vs. ≥25-<50% percentile were 418·9 ms vs. 412·7 ms in men ( P < 0·001) and 422·4 ms vs. 419·1 ms in women ( P = 0·78). Corresponding values for transferrin saturation were 417·6 ms vs. 412·6 ms in men ( P = 0·02) and 421·5 ms vs. 419·4 ms in women ( P = 0·86). The associations were not explained by inflammation and haemochromatosis genotype was not associated with QT interval length. In conclusion, increased iron stores, independent of haemochromatosis genotype and inflammation, are associated with prolongation of the QTc interval in men. This is a novel finding. In addition, the meta-analysis showed prolonged QT interval in thalassaemia major patients compared to healthy controls. [ABSTRACT FROM AUTHOR]

Iron overload cardiomyopathy remains the major cause of death in β-thalassemia (β-thal). Conventional routine screening parameters such as serum ferritin and echocardiogram (ECG) do not permit early detection of this condition. Although non-transferrin-bound iron (NTBI) is a reliable indicator for iron overload, it is still not universally available. Recently, heart rate variability (HRV), representing cardiac autonomic function, was found to be depressed in thalassemia patients. We hypothesized that HRV can be used for early detection of iron overload cardiomyopathy. Fifty patients (aged 29 ± 11 years; 31 females and 19 males) with β-thal were enrolled. The 24-hour Holter monitoring for HRV, serum ferritin, NTBI, hematological values and ECG were performed for each patient. Of the 50 patients, 29 carried β-thal major (β-TM). Non-transferrin-bound iron was weakly correlated to all time-domain HRV parameters. Low- and high-frequency domain HRV parameters were also inversely weakly correlated with NTBI. Neither HRV nor NTBI was correlated with serum ferritin. With its weak but significant correlation with NTBI, HRV may be considered to be used as a potential indicator of an iron overload condition and an early marker of cardiac involvement in patients with β-thal. [ABSTRACT FROM PUBLISHER]