Background Hereditary haemochromatosis (HH) is a common inherited disease. Abnormally increased intestinal iron absorption and accelerated recycling of iron by macrophages lead to progressive body iron accumulation and the generation of oxidative stress. In the late stages, iron overload can lead to dysfunction of the left ventricle (LV). It is believed that two‐dimensional speckle tracking echocardiography (2D STE) can evaluate LV dysfunction more accurately than conventional echocardiography. This accurate evaluation seems to be clinically important in the early stages of HH before substantial damage of the LV. Evaluation of this assessment was the purpose of this paper. Methods We prospectively enrolled 24 patients with early diagnosed HH and without any history of cardiovascular diseases; 23 healthy age‐ and sex‐matched volunteers constituted the control group. Standard echocardiographic parameters and LV rotation and strain parameters were assessed and compared between the groups. Results All echocardiographic parameters were within normal ranges, and there were no differences between the groups. 2D STE revealed significantly worse basal and apical rotation, twist and torsion values in HH patients. The peak systolic longitudinal strain was decreased in HH patients, even though the LV ejection fraction (LVEF) was normal. There were weak correlations between the iron turnover and 2D STE parameters. Conclusions 2D STE seems to be more sensitive than traditional echocardiography for detecting LV abnormalities in HH patients who are diagnosed early. The results of this study may be clinically useful, but their relevance and therapeutic implications remain to be confirmed by further studies.

IntroductionAutonomic nervous system balance can be significantly deteriorated during heart failure exacerbation. However, it is still unknown whether these changes are only the consequence of heart failure decompensation or can also predict development thereof. Objectives were to verify if simple, non-invasive autonomic parameters, such as baroreflex sensitivity and short-term heart rate variability can provide independent of other well-known clinical parameters information on the risk of heart failure decompensation in patients with left ventricular systolic dysfunction.MethodsIn 142 stable patients with left ventricular ejection fraction ≤ 40%, baroreflex sensitivity and short-term heart rate variability, as well as other well-known clinical parameters, were analyzed. During 23 ± 9 months of follow-up 19 patients were hospitalized due to the heart failure decompensation (EVENT).ResultsPre-specified cut-off values of baroreflex sensitivity (≤2.4 ms/mmHg) and low frequency power index of heart rate variability (≤19 ms2) were significantly associated with the EVENTs (hazard ratio 4.43, 95% confidence interval [CI] 1.35–14.54 and 5.41, 95% CI 1.87–15.65 respectively). EVENTs were also associated with other parameters, such as left ventricular ejection fraction, NYHA class, diuretic use, renal function, brain natriuretic peptide and hemoglobin level, left atrial size, left and right ventricular heart failure signs. After adjusting baroreflex sensitivity and low frequency power index for each of the abovementioned parameters, autonomic parameters were still significant predictors of hospitalization due to the heart failure decompensation.ConclusionSimple, noninvasive autonomic indices can be helpful in identifying individuals with increased risk of hospitalization due to the heart failure decompensation among clinically stable patients with left ventricular systolic dysfunction, even when adjusted for other well-known clinical parameters.

IntroductionThe role of implantable cardioverter-defibrillator (ICD) placement in the primary prevention of sudden cardiac death (SCD) in all consecutive patients with left ventricular ejection fraction (LVEF) ≤ 35% is still a matter of hot debate due to the fact that the population of these patients is highly heterogeneous in terms of the SCD risk. Nevertheless, reduced LVEF is still the only established criterion during qualification of patients for ICD implantation in the primary prevention of SCD, therefore identification of persons with particularly high risk among patients with LVEF ≤35% is currently of lesser importance. More important seems to be the selection of individuals with relatively low risk of SCD in whom ICD implantation can be safely postponed. The aim of the study was to determine whether well-known, non-invasive parameters, such as microvolt T-wave alternans (MTWA), baroreflex sensitivity (BRS) and short-term heart rate variability (HRV), can be helpful in the identification of low-arrhythmic risk patients with ischemic left ventricular systolic dysfunction.MethodsIn 141 patients with coronary artery disease and LVEF ≤ 35%, MTWA testing, as well as BRS and short-term HRV parameters, were analysed. During 34 ± 13 months of follow-up 37 patients had arrhythmic episode (EVENT): SCD, non-fatal sustained ventricular arrhythmia (ventricular tachycardia [VT] or ventricular fibrillation [VF]), or adequate high-voltage ICD intervention (shock) due to a rapid ventricular arrhythmia ≥200/min. LVEF, non-negative MTWA (MTWA_non-neg), BRS and low frequency power in normalized units (LFnu) turned out to be associated with the incidence of EVENT in univariate Cox analysis. The cut-off values for BRS and LFnu that most accurately distinguished between patients with and without EVENT were 3 ms/mmHg and 23, respectively. The only variable that provided 100% negative predictive value (NPV) for EVENT was negative MTWA result (MTWA_neg), but solely for initial 12 months of the follow-up; the NPVs for other potential predictors of the EVENT were lower. The cut-off values for BRS and LFnu that provide 100% NPV for EVENT during 12 and 24 months were higher: 6.0 ms/mmHg and 73 respectively, but the gain in the NPV occurred at an expense of the number of identified patients. However, the number of identified non-risk patients turned out to be higher when the predictive model included MTWA_neg and the lower cut-off values for ANS parameters: 100% NPV for 12 and 24 months of follow-up was obtained for combination MTWA_neg and BRS ≥ 3 ms/mmHg, for combination MTWA_neg and LFnu ≥ 23 100% NPV was obtained for 12 months.ConclusionWell-known, non-invasive parameters, such as MTWA, BRS and short-term HRV indices may be helpful in the identification of individuals with a relatively low risk of malignant ventricular arrhythmias among patients with ischemic left ventricular systolic dysfunction; in such persons, implantation of ICD could be safely postponed.

Background: The left ventricular (LV) hypertrophy increases the risk of heart failure. Hypertension and infiltrative cardiomyopathies are the well-known reasons of LV hypertrophy. The growing interest of scientists in this issue affects hereditary haemochromatosis (HH), which is characterised by the excess deposition of iron mostly due to HFE gene mutation. The aim of our study was to investigate the possible influence of HH on LV parameters in patients with early-diagnosed (early HH) and long-lasting and long-treated (old HH) disease. Materials and methods: Thirty nine early HH and 19 old HH patients were prospectively enrolled in the study; age-and sex-matched healthy volunteers constituted the appropriate control groups. All participants had echocardiography performed (including three-dimension volume and mass analysis); the iron turnover parameters were measured at the time of enrolment in every HH patients. Results: Echocardiographic parameters regarding to left atrium (LA), LV thickness, mass and long axis length were significantly higher, whereas LV ejection fraction was lower in early HH in comparison to healthy persons. In old HH patients the differences were similar to those mentioned before, except LV ejection fraction. The presence of hypertension in both HH groups did not influence echo parameters, as well as diabetes in old HH. The strongest correlation in all HH group was found between the time from HH diagnosis and LA, LV thickness and volumes parameters, but the correlations between iron turnover and echo parameters were non-existent. Conclusions: Hereditary haemochromatosis, not only long-lasting, but also early-diagnosed, could lead to exacerbation of LV wall thickness and cardiac hypertrophy. This effect is not simply connected with hypertension and diabetes that are frequent additional diseases in these patients, but with the time from HH diagnosis.

Background: Hereditary haemochromatosis (HH) is an inherited disease in which gene mutation leads to excessive iron absorption and accumulation in different organs, including the heart, which causes damage. Whether the age of patients with HH at the moment of their first diagnosis has an additional effect on the standard echocardiographic parameters was the aim of the study. Material and methods: We prospectively enrolled 20 HH patients, and 20 healthy age-and sex-matched volunteers. Analysis of standard echocardiographic parameters was performed and compared in subgroups of ≥50 and <50 years old (yo). Results: Comparing HH patients with healthy volunteers in ≥50 yo subgroup, significant differences were found in parameters regarding diastolic function (IVS thickness, LVM index, Em, E/Em, PV S/D, LAA index and LAV index). In the <50 yo subgroup we did not find the abovementioned differences, however LVEF appeared to be lower in the HH patients. Conclusions: Despite the lack of clinical symptoms of cardiovascular disease and the lack of deviations in the standard echocardiographic examination, there were a number of differences regarding LV diastolic function parameters in HH patients ≥50 yo, whereas differences regarding LV systolic function were more prominent in HH patients <50 yo when compared with healthy subjects.