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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.)

Deposition of amyloid in the atria (isolated atrial/cardiac amyloid) is fairly common in the aging heart. It consists of amyloid fibrils, formed both by atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) and the precursor molecule of ANP, proANP. This study examines whether amyloidogenic determinants (short peptides/amyloid forming favoring regions) exist in the sequence of NT-proBNP, the N-terminal part of proBNP, and if these determinants form amyloid-like fibrils in vitro. We have predicted a possible amyloidogenic determinant in the sequence of the NT-proBNP, and we conclusively show, after its synthesis, that it forms amyloid-like fibrils in vitro, utilizing transmission electron microscopy, X-ray diffraction, attenuated total reflectance Fourier-transform infrared spectroscopy, and polarizing microscopy. Thus, for the first time, in this study, a possible biological role is attributed to a certain, specific part of this important cardiac prohormone/natriuretic peptide, which acts as an important biomarker indicative of heart failure. Its possible direct involvement in isolated cardiac amyloidosis, atrial fibrillation, and other types of cardiac amyloidoses is indicated and discussed. Since these cardiac hormones and their prohormones play key roles in cardiovascular homeostasis through natriuresis, diuresis, vasorelaxation, and inhibition of renin and aldosterone secretion (pathophysiology of hypertension and cardiovascular regulation), we also try to suggest these specific, short peptides as possible future structural targets of efforts toward inhibiting formation of natriuretic peptide(s) amyloid., (Copyright © 2011 Wiley Periodicals, Inc.)

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.

Corrected transposition of the great arteries is a rare condition, and few patients with this abnormality survive past 50 years of age because of associated defects, or the subsequent development of atrioventricular valvular insufficiency or heart block or both. The case of our patient is of interest not only because she reached old age, but also because she lived a normal life, presenting with minor cardiac impairment and palpitations at the age of 70 years.

Background: Despite the detrimental effects of smoking on the cardiovascular system, a significant number of patients with coronary heart disease continue to smoke. We aimed to record compliance to medication and attitude towards recommended lifestyle changes in patients who suffered from myocardial infarction (MI) and continued to smoke after the coronary event., Methods: A total of 1011 consecutive patients (<75 years) with a history of MI (>6 months) were recruited during the period 2000-2003 from the outpatient cardiology clinic of a district general hospital. All patients were interviewed and blood was taken for lipid measurements. Glycosylated haemoglobin (HbA(1 c)) was also measured in all diabetics., Results: Three hundred and twenty-nine (32.5%) patients reported smoking at interview, while 338 (33.5%) were ex-smokers of whom 278 (45.8% of all smokers) had quit smoking after MI and 344 (34%) had never smoked. Persistent smokers had significantly lower high-density lipoprotein cholesterol levels than nonsmokers (1.03+/-0.28 vs. 1.09+/-0.29 mmol/l, p=0.001). Persistent smokers with diabetes had poorer glycaemic control than nonsmoker diabetic patients as indicated by HbA(1c) levels (8+/-1.7% vs. 7.2+/-1.3%, p=0.001). Fewer persistent smokers were taking hypolipidaemic drugs than nonsmokers (31% vs. 40.3%, p=0.005). Finally, persistent smokers were less frequently performing regular exercise than nonsmokers (42% vs. 51%, p=0.008)., Conclusions: Patients who remain smokers after MI have a more negative attitude towards health aspects, are less compliant with their medications, and therefore constitute a high-risk subgroup, which requires special attention and should be professionally encouraged and supported to stop smoking.

Inadvertent implantation of a pacemaker lead in the left ventricle is an uncommon complication. We report a case of a permanent pacemaker lead inadvertently placed through the left subclavian artery, across the aortic valve into the left ventricle. A chest X-ray one month after the procedure showed an unusual course of the lead and a 12-lead ECG and a transthoracic echocardiogram confirmed the diagnosis. The patient refused surgical removal and remained on full anticoagulation. No clinical events were recorded during a 3-year follow-up. In such cases we propose life-long full anticoagulation as an alternative to surgical lead extraction.