The potential impact of hereditary hemochromatosis on the heart considering the disease stage and patient age-the role of echocardiography.

Background: Hereditary hemochromatosis (HH) is a genetic disease that leads to increased iron accumulation in several organs. Cardiomyocytes are highly susceptible to this damage owing to their high iron uptake, and cardiovascular complications account for 1/3 of the deaths in the natural course of HH. Additionally, excess iron intake and associated oxidative stress may accelerate the aging of the cardiovascular system, regardless of the age of patients with HH. We aimed to investigate the role of standard and speckle-tracking echocardiography (STE) in revealing heart differences in patients with HH considering the disease stage and the patient age. Methodology: Consecutive patients with HH (n = 58) without heart pathologies (except hypertension) and 29 age- and sex-matched healthy individuals underwent echocardiography. Patients were compared according to the time since HH diagnosis (the recently diagnosed HH group [31 patients] with diagnosed HH for less than 6 months and had no more than one venesection; the medium group [11 patients] with diagnosed HH between 6 and 24 months; and the long-lasting group [16 patients] with diagnosed HH for more than 2 years) and the quartile contribution of their age. Results: Standard echocardiography revealed differences in diastolic parameters between patients with HH and controls, which were the most prominent between healthy and long-lasting HH patients. Regarding systolic function, left ventricular ejection fraction was lower in HH patients, with the most evident differences between the healthy and recently diagnosed HH patients. STE revealed additional differences in systolic parameters, with LV rotation the worst in recently diagnosed patients and its increase in patients with medium and long-lasting HH. Significantly worse peak systolic longitudinal strain values were observed in all patients with HH. Analyses of the results according to the age quartiles of patients with HH revealed that some changes ocurred earlier than expected according to age. Conclusions: Echocardiography can reveal possible heart damage in HH patients at different stages of the disease and highlight potential features of accelerated myocardial aging in these patients.

Application of Novel Technologies in Cardiac Electrotherapy to Prevent Complications.

(1) Background: Cardiac electrotherapy is developing quickly, which implies that it will face a higher number of complications, with cardiac device-related infective endocarditis (CDRIE) being the most frequent, but not the only one. (2) Methods: This is a retrospective case study followed by a literature review, which presents a patient with a rare but dangerous complication of electrotherapy, which could have been prevented if modern technology had been used. (3) Results: A 34-year-old female was admitted with suspicion of CDRIE based on an unclear echocardiographic presentation. However, with no signs of infection, that diagnosis was not confirmed, though an endocardial implantable cardioverter-defibrillator (ICD) lead was found folded into the pulmonary trunk. The final treatment included transvenous lead extraction (TLE) and subcutaneous ICD (S-ICD) implantation. (4) Conclusions: With the increasing number of implantations of cardiac electronic devices and their consequences, a high index of suspicion among clinicians is required. The entity of the clinical picture must be thoroughly considered, and various diagnostic tools should be applied. Lead dislocation into the pulmonary trunk is an extremely rare complication. Our findings align with the available literature data, where asymptomatic cases are usually effectively treated with TLE. Modern technologies, such as S-ICD, can effectively prevent lead-related problems and are indicated in young patients necessitating long-term ICD therapy.

Left Ventricle Segmental Longitudinal Strain and Regional Myocardial Work Index Could Help Determine Mitral Valve Prolapse Patients with Increased Risk of Ventricular Arrhythmias.

Mitral valve prolapse (MVP) could associate with malignant ventricular arrhythmias (VAs). Mitral annular disjunction, a putative mechanism for an arrhythmic substrate, leads to excessive mobility, stretch, and damage of some segments. Speckle tracking echocardiography (STE), with particular attention to the segmental longitudinal strain and myocardial work index (MWI), could be an indicator of the segments we aimed to check. Seventy-two MVP patients and twenty controls underwent echocardiography. Complex VAs documented prospectively after the enrollment was qualified as the primary endpoint, which was noticed in 29 (40%) patients. Pre-specified cut-off values for peak segmental longitudinal strain (PSS) and segmental MWI for basal lateral (-25%, 2200 mmHg%), mid-lateral (-25%, 2500 mmHg%), mid-posterior (-25%, 2400 mmHg%), and mid-inferior (-23%, 2400 mmHg%) segments were accurate predictors of complex VAs. A combination of PSS and MWI increased the probability of the endpoint, reaching the highest predictive value for the basal lateral segment: odds ratio 32.15 (3.78-273.8), p < 0.001 for PSS ≥ -25% and MWI ≥ 2200 mmHg%. STE may be a valuable tool for assessing the arrhythmic risk in MVP patients. Excessively increased segmental longitudinal strain with an augmented regional myocardial work index identifies patients with the highest risk of complex VAs.

Vitamin B1 Deficiency and Perimyocarditis Fulminans: A Case Study of Shoshin Syndrome in a Woman Following an Unbalanced Dietary Pattern Followed by a Literature Review.

(1) Background: vitamin B1 level depletion, known as a beriberi syndrome, can lead to severe cardiovascular complications, from which perimyocarditis fulminans is one of the most severe. (2) Methods: this is a retrospective case study that includes an adult patient with clinical presentations of acute heart failure (HF) symptoms following perimyocarditis on the grounds of thiamine deficiency. (3) Results: A 49-year-old woman presented with acute HF symptoms due to perimyocarditis. The patient suddenly developed refractory cardiogenic shock with metabolic acidosis requiring maximal medical management, including an intra-aortic balloon pump and extracorporeal membrane oxygenation. Due to additional peripheral polyneuropathy, beriberi disease was suspected after excluding other possible causes of the patient's condition. After administration of vitamin B1, clinical improvement in the patient's condition and the resolution of metabolic abnormalities were observed, which ultimately confirmed the diagnosis of Shoshin syndrome caused by the implementation of a gluten-free diet without indications for its adherence. (4) Conclusions: Fulminant beriberi disease, although considered rare, is a life-threatening condition and should always be included in the differential diagnosis of critically ill patients, notably those with malnutrition. An unbalanced diet can be detrimental and have severe consequences, i.e., perimyocarditis fulminans. However, treatment with thiamine can significantly improve the patient's cardiac function and restore hemodynamic and metabolic parameters.

A survey study of the use of a subcutaneous implantable cardioverter-defibrillator in various clinical scenarios by expert electrophysiologists in Poland.

BACKGROUND: A subcutaneous implantable cardioverter-defibrillator (S-ICD) has become a recognized alternative to a traditional transvenous implantable cardioverter-defibrillator (T-ICD). Despite the growing evidence of non-inferiority of S-ICD, there are no clear clinical guidelines for selection of either of the two available systems. The aim of the study was to analyze the decisions made in predefined typical clinical scenarios by Polish cardiologists experienced in the use of both S-ICDs and T-ICDs. METHODS: A group of 30 experts of cardiac electrotherapy experienced in the use of S-ICDs was recruited and invited to participate in a web-based anonymous survey. The survey questions regarded the proposed therapy in various but typical clinical scenarios. RESULTS: From the invited 30 experts representing 18 clinical centers, 25 completed the survey. 72% of them declared that the number of S-ICDs implanted at their center during the preceding 12 months exceeded 10, and 40% - that it was over 20. Rates of responders preferring S-ICD or T-ICD in various clinical scenarios are reported and discussed in detail. CONCLUSIONS: Significant divergence of opinion exists among Polish experts regarding the use of a subcutaneous cardioverter-defibrillator. It is especially pronounced on the issue of the use of the system in middle-age patients, in case of complications of the hitherto ICD therapy, or the need of upgrading the existing cardiac implantable electronic device.

Eligibility of patients with temporary paced rhythm for a subcutaneous implantable cardioverter-defibrillator.

BACKGROUND: A concomitant use of a pacemaker and a subcutaneous implantable cardioverter-de-fibrillator (S-ICD) may be required in some patients. AIMS: Our study aimed to evaluate the influence of permanent cardiac pacing on the morphology of the QRS complex in the context of S-ICD screening. METHODS: One hundred patients with cardiac electronic implantable devices (CIEDs) were included in whom S-ICD screening could be performed both during intrinsic and paced rhythm. RESULTS: The positive result of screening during spontaneous rhythm for at least one vector (in both supine and standing positions) was obtained in 80% and for 2 vectors in 59% of patients. Positive screening during paced rhythm for at least one vector was recorded in 36% of patients (78% right ventricular and 22% biventricular pacing) and for 2 vectors in 15% of patients (93% right ventricular and 7% biventricular pacing). At least one vector acceptable during both types of rhythm and in both positions was recorded in 23% of patients and at least 2 vectors in 8% of patients. CONCLUSIONS: The use of S-ICD in patients with paced ventricular rhythm is associated with a serious risk of inappropriate sensing due to different QRS morphology during intrinsic and paced rhythm, and it is particularly high in patients in whom periods of spontaneous rhythm interchange with periods of ventricular pacing. That risk has been hardly acknowledged in available reports, but according to our data, it is significant, and therefore it should be considered during S-ICD screening.

Real-Life Inter-Rater Variability of the PRAETORIAN Score Values.

(1) Background: The PRAETORIAN score is a tool developed for postoperative evaluation of the position of subcutaneous implantable cardioverter-defibrillator systems. The aim of our study was to evaluate the real-life inter-rater variability of the PRAETORIAN score, based on chest radiographs of S-ICD patients reviewed by independent clinical raters. (2) Methods: Postoperative chest X-rays of patients that underwent S-ICD implantation were evaluated by five clinical raters who gave values of the PRAETORIAN score. Ratings were then compared in a fully crossed manner to determine the inter-rater variability of the attributed scores. (3) Results: In total, 87 patients were included in the study. In the case of the most important final risk category of the PRAETORIAN score, the mean Light's kappa was 0.804, the Fleiss' kappa was 0.249, and the intraclass correlation was 0.38. The final risk category was identically determined by all five raters in 75.86% of patients, by four raters in 14.94%, and by three raters in 9.20% of patients. (4) Conclusions: The overall inter-rater variability of the PRAETORIAN score in a group of electrophysiologists experienced in S-ICD implantation, yet previously naive to the PRAETORIAN score, and self-trained in its utilization, was only modest in our study. Appropriate use of the score might require training of clinical raters.

New-Onset Atrial Fibrillation in Acute Myocardial Infarction Is a Different Phenomenon than Other Pre-Existing Types of That Arrhythmia.

(1) Background: Atrial fibrillation (AF) in acute myocardial infarction (AMI) could worsen the prognosis. Yet, there is no definitive answer to whether new-onset AF (NOAF) is a more aggravating diagnosis than other types of that arrhythmia. The purpose of our study was to compare in-hospital clinical course and outcomes of NOAF patients contrary to patients with other pre-existing types of AF. (2) Methods: AMI patients hospitalized in the high-volume cardiological center within 2017−2018 were included in the study. NOAF was noticed in 106 (11%) patients, 95 (10%) with an AF history and AF during AMI formed the AF group, 60 (6%) with an AF history but without AF during AMI constituted the Prior-AF group, and 693 (73%) patients were without an AF before and during AMI. Medical history, routinely monitored clinical parameters, and in-hospital outcomes were analyzed between the groups. (3) Results: NOAF patients, contrary to others, initially had the highest high-sensitivity troponin I (hsTnI), B-type natriuretic peptide (BNP), C-reactive protein (CRP), and glucose levels, and the lowest potassium concentration, with the worst profile of changes for that parameter within the first four days of hospitalization. NOAF patients had the highest rate of ST-elevated AMI (40%), the longest hospitalization (p < 0.001), and the highest in-hospital mortality (p < 0.001). Not NOAF, but other AF groups (AF and Prior-AF groups) were more burdened with the previous comorbidities. (4) Conclusions: NOAF could be a distinct phenomenon in AMI patients, identifying those with the worst clinical in-hospital course and outcomes as compared to other types of AF.

Exercise-Induced Atrial Remodeling in Female Amateur Marathon Runners Assessed by Three-Dimensional and Speckle Tracking Echocardiography.

Endurance athletes have an increased risk of atrial remodeling and atrial arrhythmias. However, data regarding atrial adaptation to physical exercise in non-elite athletes are limited. Even less is known about atrial performance in women. We aimed to elucidate exercise-induced changes in atrial morphology and function in female amateur marathon runners using three-dimensional (3D) echocardiography and two-dimensional (2D) speckle tracking echocardiography (STE). The study group consisted of 27 female (40 ± 7 years) amateur athletes. Right (RA) and left atrial (LA) measures were assessed three times: 2-3 weeks before the marathon (stage 1), immediately after the run (stage 2), and 2 weeks after the competition (stage 3). Directly after the marathon, a remarkable RA dilatation, as assessed by RA maximal volume (RAVmax, 31.3 ± 6.8 vs. 35.0 ± 7.0 ml/m2; p = 0.008), with concomitant increase in RA contractile function [RA active emptying fraction (RA active EF), 27.7 ± 8.6 vs. 35.0 ± 12.1%; p = 0.014; RA peak atrial contraction strain (RA PACS) 13.8 ± 1.8 vs. 15.6 ± 2.5%; p = 0.016] was noticed. There were no significant changes in LA volumes between stages, while LA active EF (34.3 ± 6.4 vs. 39.4 ± 8.6%; p = 0.020), along with LA PACS (12.8 ± 2.1 vs. 14.9 ± 2.7%; p = 0.002), increased post race. After the race, an increase in right ventricular (RV) dimensions (RV end-diastolic volume index, 48.8 ± 11.0 vs. 60.0 ± 11.1 ml/m2; p = 0.001) and a decrease in RV function (RV ejection fraction, 54.9 ± 6.3 vs. 49.1 ± 6.3%; p = 0.006) were observed. The magnitude of post-race RV dilatation was correlated with peak RA longitudinal strain deterioration (r = -0.56, p = 0.032). The measured parameters did not differ between stages 1 and 3. In female amateur athletes, apart from RV enlargement and dysfunction, marathon running promotes transient biatrial remodeling, with more pronounced changes in the RA. Post-race RA dilatation and increment of the active contraction force of both atria are observed. However, RA reservoir function diminishes in those with post-race RV dilation.

Baroreflex sensitivity but not microvolt T-wave alternans can predict major adverse cardiac events in ischemic heart failure.

BACKGROUND: Major adverse cardiovascular events (MACE) constitutes the main cause of morbidity and mortality in ischemic heart failure (HF) patients. The prognostic value of the autonomic nervous system parameters and microvolt T-wave alternans (MTWA) in this issue has not been identified to date. The aim herein, was to assess the usefulness of the abovementioned parameters in the prediction of MACE in HF patients with left ventricular systolic dysfunction of ischemic origin. METHODS: Baroreflex sensitivity (BRS), heart rate variability (HRV), MTWA and other well-known clinical parameters were analyzed in 188 ischemic HF outpatients with left ventricular ejection fraction (LVEF) ≤ 50%. During 34 (14-71) months of follow-up, 56 (30%) endpoints were noted. RESULTS: Univariate Cox analyses revealed BRS (but not HRV), MTWA, age, New York Heart Association functional class III, LVEF, implantable cardioverter-defibrillator presence, use of diuretics and antiarrhythmic drugs, diabetes, and kidney insufficiency were defined as significant predictors of MACE. Pre-specified cut-off values for MACE occurrence for the aforementioned continuous parameters (age, LVEF, and BRS) were: ≥ 72 years, ≤ 33%, and ≤ 3 ms/mmHg, respectively. In a multivariate Cox analysis only BRS (HR 2.97, 95% CI 1.35-6.36, p < 0.006), and LVEF (HR 1.98, 95% CI 0.61-4.52, p < 0.038) maintained statistical significance in the prediction of MACE. CONCLUSIONS: Baroreflex sensitivity and LVEF are independent of other well-known clinical parameters in the prediction of MACE in patients with HF of ischemic origin and LVEF up to 50%. BRS ≤ 3 ms/mmHg and LVEF ≤ 33% identified individuals with the highest probability of MACE during the follow-up period.

Electromagnetic Field Associated With Dermoscope Magnets May Affect the Safety of Cardiac Implanted Electronic Devices Patients.

Dermoscopy is currently used as an auxiliary tool in general dermatology. Since some commercially available dermoscopes have built-in magnets, electromagnetic interference (EMI) may occur when examining cardiac implantable electronic devices (CIED) patients. The aim of the study was to create maps of electromagnetic fields defining a safe distance in terms of EMI. The study was performed in laboratory conditions using measuring equipment specially designed for this purpose. The following dermoscopes have been tested: Illuco IDS-1100, Visiomed Luminis, Visiomed Luminis 2, Heine NC2 with and without a contact plate, DermLite DL4, and DermLite Handyscope. Measurements were made for the following set of lift-off distances: 5, 10, 20, 30, 40, 50, and 150 mm. Each 2D scan consisted of 10-line scans shifted from each other by 10 mm. The strength of the magnetic field decreased with the distance from the faceplate. The distribution of the magnetic field differed depending on the position of the magnets. The highest magnetic field was recorded in the center of the Heine NC2 faceplate (up to 8 mT). In most cases, at a distance of 10 mm, the magnetic field strength was measured below 1 mT, with the exception of Heine NC2 and Heine NC2 with a contact plate. All tested dermoscopes generated a magnetic field of <1 mT at the distance of 20 mm. The use of dermoscopes with built-in magnets may affect the functioning of CIEDs, and the impact may vary depending on the type of dermoscope.

Comprehensive Use of Routine Clinical Parameters to Identify Patients at Risk of New-Onset Atrial Fibrillation in Acute Myocardial Infarction.

(1) Background: New-onset atrial fibrillation (NOAF) is a significant complication of acute myocardial infarction (AMI). Our study aimed to investigate whether routinely checked clinical parameters aid in NOAF identification in modernly treated AMI patients. (2) Patients and methods: Patients admitted consecutively within 2017 and 2018 to the University Clinical Centre in Gdansk (Poland) with AMI diagnosis (necrosis evidence in a clinical setting consistent with acute myocardial ischemia) were enrolled. Medical history and clinical parameters were checked during NOAF prediction. (3) Results: NOAF was diagnosed in 106 (11%) of 954 patients and was significantly associated with in-hospital mortality (OR 4.54, 95% CI 2.50-8.33, p < 0.001). Age, B-type natriuretic peptide (BNP), C-reactive protein (CRP), high-sensitivity troponin I, total cholesterol, low-density lipoprotein cholesterol, potassium, hemoglobin, leucocytes, neutrophil/lymphocyte ratio, left atrium size, and left ventricular ejection fraction (LVEF) were associated with NOAF in the univariate logistic analysis, whereas age ≥ 66 yo, BNP ≥ 340 pg/mL, CRP ≥ 7.7 mg/L, and LVEF ≤ 44% were associated with NOAF in the multivariate analysis. (4) Conclusions: NOAF is a multifactorial, significant complication of AMI, leading to a worse prognosis. Simple, routinely checked clinical parameters could be helpful indices of this arrhythmia in current invasively treated patients with AMI.

Electrocardiographic Changes in Male and Female Amateur Marathon Runners: A Comparison Study.

Physical training is gaining popularity among amateurs. Males and females exhibit different cardiac adaptation to exercise. The aim of the study was to compare the incidence of electrocardiographic abnormalities before and after the marathon between sexes. 12-lead electrocardiogram was performed in 40 male (39±8 years) and 27 female (40±7 years) amateur runners: 2-3 weeks before (Stage 1) and immediately after (Stage 2) the marathon. Abnormalities in the resting (Stage 1) and exercise (Stage 2) electrocardiograms were compared between sexes. At rest left atrial enlargement was more frequent in females than males (48 vs. 20%; p<0.05). The incidence of right atrial enlargement was significantly more common at Stage 2 than 1, both in men (43 vs. 0%; p<0.001) and in women (48 vs. 4%; p=0.001). Significant increase of P-wave amplitude was found in male runners after the marathon (0.12±0.05 vs. 0.21±0.09 mV; p<0.001 Stage 1 vs. 2), but was absent in females. QTc prolongation was observed in both sexes, however to a higher degree in males (p<0.05 for the interaction stage and sex). Although both male and female amateur marathon runners exhibit abnormalities in resting and exercise electrocardiograms, men present more exercise-induced electrocardiographic changes, which might indicate a higher propensity for post-marathon arrhythmias. Electrocardiographic screening in amateurs should be considered.

Cardiovascular events in patients with familial hypercholesterolemia and hyperlipoproteinaemia (a): Indications for lipoprotein apheresis in Poland.

BACKGROUND: Lipoprotein apheresis (LA) is a safe method of reducing atherogenic lipoproteins and improving cardiovascular (CV) outcomes. We aimed to assess the reductions in low-density lipoprotein cholesterol (LDL-C) and lipoprotein (a) [Lp(a)] levels in patients undergoing regular LA therapy and to evaluate its influence on the incidence rate of adverse cardiac and vascular events (ACVE) and major adverse cardiac events (MACE). METHODS: A longitudinal study in Poland evaluated the prospective and retrospective observational data of 23 patients with hyperlipoproteinaemia (a) [hyper-Lp(a)] and familial hypercholesterolemia (FH), undergoing 1014 LA sessions between 2013 and 2020. Their pre- and post-apheresis LDL-C and Lp(a) levels were assessed to calculate the acute percent reductions. The time period used to evaluate annual rates of ACVE and MACE before and after initiation of LA was matched in each patient. RESULTS: The pre-apheresis LDL-C and Lp(a) concentrations were 155 (107-228) (mg/dL) (median and interquartile range) and 0.56 (0.14-1.37) (g/L), respectively. LA therapy resulted in a reduction of LDL-C to 50 (30-73.5) (mg/dL) and of Lp(a) to 0.13 (0.05-0.34) (g/L), representing a percent reduction of 70.0% and 72.7% for LDL-C and Lp(a), respectively. We found a significant reduction in the annual rate of ACVE (0.365[0.0-0.585] vs (0.0[0.0-0.265]; P = .047) and MACE (0.365[0.0-0.585] vs 0.0[0.0-0.265]; P = .031). CONCLUSIONS: The findings of our study indicate that LA treatment in patients with hyperlipoproteinaemia (a) and FH on maximally tolerated lipid lowering therapies leads to a substantial reduction in LDL-C and Lp(a) concentrations and lowers CV event rates in Polish patients.