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Background: β-thalassemia major is a unique disease characterized by early severe diastolic dysfunction, due to iron myocardial deposition alone, while left ventricular systolic dysfunction and failure seem to be multifactorial in aitiology., Objectives: The purpose of this study was to investigate left ventricular diastolic dysfunction using a new echo index as speckle tracking in comparison with the conventional methods., Material and Methods: Eighty-eight consecutive patients (38 male, 50 female) aged 36 ± 8.2 yr with β-thalassemia major and preserved LV ejection fraction (LVEF>55%) were studied. Patients were divided into two groups according to the E mitral/E mitral annulus ratio (E/E'): group A patients with E/E' ratio ≤8 and group B patients with E/E' >8. Cutoff value of eight was used to separate patients with normal and abnormal diastolic function. All subjects were studied thoroughly by tissue Doppler echocardiography as also by 2D left ventricular and atrial strain imaging 2-4 d following blood transfusion. Blood samples were also taken for plasma BNP measurements at the same time., Results: Left atrial volumes(LAV max, LAV min) as also left atrial index were significantly higher in patients with diastolic dysfunction compared with patients without diastolic dysfunction(LAV max: 57.6 ± 19.4 vs. 71.3 ± 22.9, P < 0.01,LAV min: 20.2 ± 11.4 vs. 33.9 ± 18, P < 0.01, LAVI: 37.66 ± 12.18 vs. 47.13 ± 14.77, P < 0.01). Radial 2D strain (RS) and peak atrial 2D strain (AS) were significantly reduced in patients with suspected diastolic dysfunction compared with patients without diastolic dysfunction (RS: 43.48 ± 13.92 vs. 35.58 ± 11.32, P < 0.05; AS: 36.36 ± 8.45 vs. 29.85 ± 9.25, P < 0.01). Using ROC analysis, peak atrial 2D strain at a cutoff of 41.1 cm/s was highly accurate (AUC: 0.66, P < 0.05 in ruling out diastolic dysfunction (E/E'<8) with a sensitivity of 90% and a specificity of 81%., Conclusions: B-thalassemic major patients with preserved left ventricular systolic function had impaired left atrial function at the longitudinal axis and left ventricular function at the radial axis. The new echo markers have better prognostic value than the traditional echo indexes in detecting latent diastolic dysfunction in β-thalassemia major, earlier than E/E' ratio., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: The early diagnosis and treatment of heart failure in beta-thalassaemic patients is related to survival. Iron alone or in combination with other factors causes diastolic dysfunction, which usually precedes systolic dysfunction. NT-proBNP is a sensitive biomarker of ventricular dysfunction., Aim: To evaluate NT-proBNP in non heart failure beta-thalassaemic patients., Methods: Fifty-two beta-thalassaemia major patients (mean age: 27.2+/-12.5 years) with normal LV systolic function, underwent NT-proBNP measurement and a thorough Doppler-echocardiographic and pulsed tissue-Doppler study, 4 days following blood transfusion. Fifty-two age matched healthy controls were also studied., Results: NT-proBNP and E/E' ratio were increased in thalassaemic patients compared with controls [(469+/-171 vs 262+/-51 pmol/l, p<0.001) and (10.8+/-4.0 vs 6.6+/-1.1, p<0.001)] and were well correlated (r: 0.54, p<0.01). Although NT-proBNP levels were increased in patients with higher E/E' ratios (E/E' <8: 354+/-119, 8-15: 516+/-177, >15: 565+/-114 pmol/l, ANOVA p: 0.002) this increase only became statistically significant in the 3rd decade of life, while E/E' ratio increased in the 4th decade., Conclusion: NT-proBNP increases in beta-thalassaemia major patients and is related to age and LV diastolic dysfunction. NT-proBNP appears to be an early biomarker of LV diastolic dysfunction, compared with the conventional Echo-Doppler indexes.