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.

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.

Vascular Aging and Damage in Patients with Iron Metabolism Disorders.

Vascular aging is a physiological, multifactorial process that involves every type of vessel, from large arteries to microcirculation. This manifests itself as impaired vasomotor function, altered secretory phenotype, deteriorated intercellular transport function, structural remodeling, and aggravated barrier function between the blood and the vascular smooth muscle layer. Iron disorders, particularly iron overload, may lead to oxidative stress and, among other effects, vascular aging. The elevated transferrin saturation and serum iron levels observed in iron overload lead to the formation of a non-transferrin-bound iron (NTBI) fraction with high pro-oxidant activity. NTBI can induce the production of reactive oxygen species (ROS), which induce lipid peroxidation and mediate iron-related damage as the elements of oxidative stress in many tissues, including heart and vessels' mitochondria. However, the available data make it difficult to precisely determine the impact of iron metabolism disorders on vascular aging; therefore, the relationship requires further investigation. Our study aims to present the current state of knowledge on vascular aging in patients with deteriorated iron metabolism.

Chronic Fatigue Syndrome in Patients with Deteriorated Iron Metabolism.

Fatigue is a common, non-specific symptom that often impairs patients' quality of life. Even though fatigue may be the first symptom of many serious diseases, it is often underestimated due to its non-specific nature. Iron metabolism disorders are a prominent example of conditions where fatigue is a leading symptom. Whether it is an iron deficiency or overload, tiredness is one of the most common features. Despite significant progress in diagnosing and treating iron pathologies, the approach to chronic fatigue syndrome in such patients is not precisely determined. Our study aims to present the current state of knowledge on fatigue in patients with deteriorated iron metabolism.

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.