Silesian Center for Heart Disease

The aim of the study was to comprehensively characterize the population of hypertrophic cardiomyopathy (HCM) patients in Poland in 2016-2021, including the analysis of annual incidence, prevalence, comorbidities, and therapeutic paths. A population-based cross-sectional study was conducted using data from the Polish National Health Fund database. The analysis included a registered annual incidence and prevalence rates, mortality, prognosis, and data on patients pathways in the healthcare system. In 2016, the registered annual incidence of HCM diagnosis in Poland was 1,494 cases (3.89/100,000), decreasing to 808 cases (2.11/100,000) in 2021. Despite the decline in new diagnoses, the registered prevalence of HCM increased from 13,271 cases (34.53/100,000) in 2016 to 13,880 cases (36.23/100,000) in 2021, indicating improved patient survival. Annual mortality rose from 824 deaths (2.14/100,000) in 2016 to 1,022 deaths (2.67/100,000) in 2021, with the highest mortality observed in older adults (60+ years). The study highlights significant underdiagnosis of HCM in Poland. Mortality in HCM population remains high, underscoring the need for improvement in earlier detection and comprehensive care strategies in Polish healthcare system.

Introduction: Osteoprotegerin (OPG) is recognized as an emerging biomarker for atherosclerosis. We hypothesized that atherosclerotic lesions localized across multiple vascular beds would result in greater elevations in OPG levels in the blood. Therefore, our study aimed to assess serum OPG levels and their confounding factors in patients with hemodynamically significant multivessel atherosclerosis in varying locations. Subjects and Methods: A case-control study included 222 selected outpatients aged 50 years or older (46.4% women) with atherosclerosis confirmed by imaging (Doppler ultrasound and CT angiography) treated at a single angiology clinic. Data concerning age, smoking status, comorbidity (hypertension, diabetes mellitus, history of stroke, myocardial infarction, coronary revascularization procedures), medication, lipid profile, serum creatinine, and homocysteine levels were retrieved from medical records. Additionally, serum OPG levels were measured. Patients were divided according to serum OPG levels into terciles and the number of involved vascular beds [carotid artery disease, coronary heart disease (CHD), lower-extremity peripheral artery disease (PAD), abdominal aorta aneurysm (AAA)]. Results: The distribution of carotid artery disease, CHD, PAD, and AAA did not differ across the OPG terciles. Additionally, we did not observe differences in OPG levels between specific and multiple locations of atherosclerotic lesions. Subjects with the highest OPG levels were the oldest (75.0 ± 8.4 vs. 69.8 ± 7.1 years in the lowest tercile; p < 0.001) and were characterized by the worst kidney function (eGFR 60.8 ± 16.8 vs. 74.1 ± 13.5 mL/min/1.73 m2; p < 0.001). Conclusions: The serum OPG level did not reveal the specific location of atherosclerosis. Impaired renal function appears to be the primary determinant of serum OPG levels and a key confounder, complicating the interpretation of serum OPG as a biomarker of atherosclerosis.

Uhl's anomaly is an extremely rare congenital heart defect, with approximately 100 cases reported since its first description in 1952 by Dr Henry Uhl. Characterized by partial or complete absence of the right ventricular myocardium, it leads to progressive right ventricle dilation and right heart failure. Due to its rarity, there is no consensus regarding its surgical or medical management. We present a case of an 18-year-old male patient with Uhl's anomaly diagnosed in early infancy, managed by palliative Glenn shunt. This report provides insight into management of this rare condition.

Aortic diseases, including thoracic and abdominal aneurysms as well as aortic dissections, represent life-threatening vascular disorders characterized by progressive wall degeneration and inflammation. Increasing evidence indicates that oxidative stress is a central driver of aortic pathology through the induction of DNA damage in vascular smooth muscle cells and endothelial cells. Oxidative DNA lesions activate the DNA damage response (DDR), a highly coordinated network of damage sensors, signaling kinases, and repair effectors that determines cell fate decisions such as DNA repair, apoptosis, or cellular senescence. In aortic tissue, persistent or dysregulated DDR signaling contributes to chronic inflammation, extracellular matrix degradation, and loss of vascular integrity. Key molecular regulators, including base excision repair enzymes OGG1 and APE1, as well as DDR mediators such as ATM, ATR, p53, PARP, and NOTCH1, integrate oxidative stress signals with pro-inflammatory and pro-degenerative pathways. Aberrant activation of these mechanisms promotes vascular smooth muscle cell VSMC phenotypic switching from contractile to synthetic phenotype, endothelial dysfunction, and senescence-associated secretory responses, thereby accelerating aortic wall weakening and aneurysm progression. This review highlights the mechanistic links between oxidative stress-induced DNA damage, DDR pathway activation, and vascular remodeling in aortopathies. A deeper understanding of these molecular interactions may uncover novel biomarkers and therapeutic targets aimed at limiting inflammation, preserving genomic stability, and preventing catastrophic aortic events. This work represents a narrative review and therefore has inherent limitations in terms of systematic literature search and selection.

Cyclin-dependent kinase 4/6 inhibitors (CDK4/6i) are the standard of care in advanced breast cancer. While Ki67 is prognostic in early-stage disease, including adjuvant CDK4/6i, its role in metastatic setting is unclear. This study assessed Ki67’s prognostic value in advanced breast cancer treated with CDK4/6i. We conducted a retrospective analysis of patients with advanced breast cancer receiving CDK4/6i at our cancer center between 2018 and 2023. The study included 368 patients with Ki67 results. The median age was 63 years (IQR 52–70.5). 283 patients (76.9%) received CDK4/6i in the first-line. With respect to biopsy timing, Ki67 expression was evaluated in tissue samples from 319 patients in the metastatic setting and 49 patients in the early breast cancer setting. In terms of biopsy site, Ki67 was measured from the breast tumor in 235 patients, from regional lymph nodes in 26 patients, and from various metastatic sites in 107 patients. Ki67, as a continuous variable, correlated with PFS and OS. Specifically, each unit increase in Ki67 was associated with a 1% reduction in PFS and OS. Median PFS in patients with lowKi67 was 33.0 months vs. 22.0 months for highKi67 (p = 0.008). Two-year PFS was 68.3% for lowKi67, vs. 48.4% for highKi67. Median OS was 49.7 months for lowKi67 vs. 39.8 months for highKi67 (p = 0.027). Ki67 appears to be a prognostic factor in advanced breast cancer patients treated with CDK4/6i. Patients with low Ki67 had favorable prognosis.

Introduction and aim. The most common metabolic disorders include type 2 diabetes (T2DM) and obesity. Their prevalence has increased in recent years. Due to their widespread prevalence and the fact that they increase the risk of cardiovascular disease, morbidity, and mortality, they pose a significant economic burden to the healthcare system. In this review, we will focus primarily on the role of gut hormone signaling produced by enteroendocrine cells (EECs), which are part of the gut-brain axis. Furthermore we will summarize applications of these mechanisms in novel therapies for T2DM and obesity. Material and method. Literature data analysis was performed using the following databases: PubMed (MEDLINE), Scopus, Web of Science and Google Scholar. The review included articles in Polish and English published between 2000 and 2024. Analysis of the literature. EECs are specialized transepithelial cells present throughout the intestine. The best-studied EEC subtype is the L cell, which secretes glucagon-like peptide-1 (GLP-1). GLP-1 regulates insulin secretion and contributes to satiety by increasing insulin secretion and inhibiting glucagon secretion. Significant progress in the use of intestinal hormones in the treatment of T2DM and obesity has led to the development of effective therapies for both of these conditions, such as GLP-1 analogs. Conclusion. The growing understanding of biochemical processes, hormonal signaling, and the development of new technologies contribute to the continuation of research on new, more effective therapies that use mechanisms of action of the gut-brain axis. Despite these achievements, the need for new and more effective treatments is constantly growing, and requires innovative strategies and their potential combination with existing therapies.