One of the main causes of heart failure is cardiomyopathies. Among them, the most common is hypertrophic cardiomyopathy (HCM), characterized by thickening of the left ventricular muscle. This article focuses on HCM and other cardiomyopathies with myocardial hypertrophy, including Fabry disease, Pompe disease, and Danon disease. The genetics and pathogenesis of these diseases are described, as well as current and experimental treatment options, such as pharmacological intervention and the potential of gene therapies. Although genetic approaches are promising and have the potential to become the best treatments for these diseases, further research is needed to evaluate their efficacy and safety. This article describes current knowledge and advances in the treatment of the aforementioned cardiomyopathies.

Background: The prevalence of portopulmonary hypertension (PoPH) is relatively low; however, its presence significantly worsens patients’ prognosis. When diagnosed, PoPH can be effectively treated, and specific therapies can lead to a substantial reduction in pulmonary circulation pressure, facilitating the safe performance of liver transplantation. Echocardiography is recommended as a first-line method for the non-invasive diagnosis of pulmonary hypertension and serves as a valuable screening tool for patients being evaluated for liver transplantation (LT). The objective of this study was to thoroughly assess the occurrence of echocardiographic signs indicative of pulmonary hypertension and hepatopulmonary syndrome (HPS) in candidates for LT. We assumed that our analysis also made it possible to assess how frequently these candidates require further invasive diagnostics for pulmonary hypertension at specialized centers and how often they may need targeted treatment for pulmonary arterioles as a bridge to transplantation, which could improve patient outcomes. Additionally, this study included a comprehensive review of the current literature. Methods: All LT candidates underwent standardized transthoracic echocardiography and contrast evaluation to identify intrapulmonary vascular shunts. Results: A total of 152 liver transplantation candidates (67 women, mean age 50.6 years) were included in the analysis. The estimated echocardiographic probability of pulmonary hypertension was classified as high in only one patient. However, 63 patients exhibited the visualization of microbubbles in the left heart chambers after an average of six cardiac cycles (ranging from three to nine cycles) following their appearance in the right heart. Conclusions: Our analysis shows that the features of PoPH and a high probability of PH were very rare in the LT candidates, and echocardiographic signs suggestive of hepatopulmonary syndrome were more prevalent. Liver transplant candidates need screening for PoPH and HPS, as both PoPH and HPS significantly worsen their prognosis, but specific PH treatment as a bridge to transplantation improves PoPH patients’ survival.

Kidney transplant recipients are reported to have low levels of physical activity compared to the general population. This population is burdened by high prevalence of both traditional and non-traditional cardiovascular risk factors. Physical activity has multiple known health benefits, including reduction of cardiovascular disease risk factors, which are the most common cause of morbidity and mortality in this group. Sedentary lifestyle has been associated with poorer long-term graft outcomes and inferior quality of life. Lack of specific guidelines regarding exercise post-transplantation has been cited as one of the barriers to physical activity, alongside comorbidities and immunosuppressive drugs’ side effects. Exercise interventions, leading to improved physical functioning both pre- and post-transplant, have been demonstrated to lead to improved muscle strength, overall physical functioning, as well as cardiorespiratory performance. Currently available data on physical activity in kidney transplant recipients and its role in long term management of these patients remains limited, but promising results regarding its impact on long-term outcomes highlight the need for further research and development of more precise guidelines and educational programmes for both healthcare professionals and kidney transplant recipients.

Background: Decreased hemoglobin concentration was reported to predict long term prognosis in patients various cardiovascular diseases including congestive heart failure and coronary artery disease. We hypothesized that hemoglobin levels may be useful for post discharge prognostication after the first episode of acute pulmonary embolism. Therefore, the aim of the current study was to evaluate a potential prognostic value of a decreased hemoglobin levels measured at admission due to the first episode of acute PE for post discharge all cause mortality during at least 2 years follow up. Methods: This was a prospective, single-center, follow-up, observational, cohort study of consecutive survivors of the first PE episode. Patients were managed according to ESC current guidelines. After the discharge, all PE survivors were followed for at least 24 months in our outpatient clinic. Results: During 2 years follow-up from the group of 402 consecutive PE survivors 29 (7.2%) patients died. Non-survivors were older than survivors 81 years (40–93) vs. 63 years (18–97) p < 0.001 presented higher sPESI 2 (0–4) vs. 1 (0–5), p < 0.001 driven by a higher frequency of neoplasms (37.9% vs. 16.6%, p < 0.001); and had lower hemoglobin (Hb) level at admission 11.7 g/dL (6–14.8) vs. 13.1 g/dL (3.1–19.3), p < 0.001. Multivariable analysis showed that only Hb and age significantly predicted all cause post-discharge mortality. ROC analysis for all cause mortality showed AUC for hemoglobin 0.688 (95% CI 0.782–0.594), p < 0.001; and for age 0.735 (95% CI 0.651–0.819) p < 0.001. A group of 59 subjects with hemoglobin < 10.5 g/dL showed mortality rate of 16.9% (OR for mortality 4.19 (95% CI 1.82–9.65), p-value < 0.00, while among 79 patients with Hb > 14.3 g/dL only one death was detected. Interestingly, patients in age > 64 years hemoglobin levels < 13.2 g/dL compared to patients in the same age but with >13.2 g/dL showed OR 3.6 with 95% CI 1.3–10.1 p = 0.012 for death after the discharge. Conclusions: Lower haemoglobin measured in the acute phase especially in patients in age above 64 years showed significant impact on the prognosis and clinical outcomes in PE survivors.

Introduction and puropse. Atrial fibrillation (AF) is the most common sustained arrhythmia worldwide, closely associated with rising obesity rates and sedentary lifestyles. This review evaluates the evidence linking obesity, physical activity, and weight reduction with AF development, progression, and management. Material and methods. A comprehensive literature review was conducted using PubMed and Google Scholar, prioritizing randomized trials, meta-analyses, and recent studies published within the past eight years addressing obesity, physical activity, and weight loss in AF. Brief description of the state of knowledge. Obesity is a major risk factor for atrial fibrillation, with each 5-unit BMI increase linked to a 28–30% rise in risk. Higher BMI is associated with increased AF incidence, faster progression, and recurrence rates up to 50% after ablation in severe obesity. Moderate physical activity contributes to lower risk of AF and improved rhythm control, while high-intensity endurance exercise may elevate AF risk, particularly in middle-aged men. Sustained weight loss of at least 10% significantly reduces AF burden, recurrence rates, and symptom severity. Bariatric surgery has been shown to decrease AF incidence in severely obese patients, and long-term weight stability enhances these benefits. Anticoagulant efficacy remains stable across BMI ranges, especially rivaroxaban, which shows favorable profiles in obese patients. Conclusions. Current evidence supports the integration of lifestyle interventions such as targeted weight loss and individualized physical activity into AF management. These strategies offer significant benefits to AF risk, arrhythmia control, and overall outcomes, supporting routine implementation in clinical practice.

Wstęp: Gaz w żyle wrotnej (hepatic portal venous gas – HPVG) jest rzadkim, lecz alarmującym objawem u pacjentów pediatrycznych, historycznie wiążącym się ze śmiertelnością sięgającą 75%. Jednak dzięki postępowi w technikach obrazowania można stwierdzić, że w wielu przypadkach ma on charakter przejściowy i łagodny. W badaniu skupiono się na rosnącej roli ultrasonografii w diagnostyce dzieci z HPVG, podkreślając jej znaczenie w praktyce klinicznej.Materiał i metody: Przeprowadzono przegląd literatury w bazach PubMed i Google Scholar. Użyto haseł: „hepatic portal venous gas”, „pediatrics”, „ultrasonography”, „diagnostic imaging”, „necrotizing enterocolitis” oraz ich wariantów.Stan wiedzy: HPVG można wykryć za pomocą różnych metod obrazowania. Spośród nich ultrasonografia wyróżnia się wysoką czułością, brakiem promieniowania jonizującego oraz możliwością uzyskania wyników w czasie rzeczywistym. U pacjentów pediatrycznych rozszerzone badanie ultrasonograficzne pozwala nie tylko na wykrycie HPVG, lecz także na ocenę ryzyka poprzez analizę wzoru rozkładu gazu i innych ultrasonograficznych markerów związanych z gorszym rokowaniem. Ponadto ultrasonografia ułatwia zawężenie diagnostyki różnicowej poprzez śledzenie przepływu gazu w skomplikowanych gałęziach układu wrotnego. Co istotne, połączenie wykrywania HPVG za pomocą ultrasonografii z kryteriami Gordona znacząco poprawia dokładność diagnostyczną martwiczego zapalenia jelit, osiągając 86% swoistości i 90% czułości. Podkreśla to rosnące znaczenie ultrasonografii w opiece pediatrycznej.Wnioski: Ultrasonografia jest skutecznym, nieinwazyjnym narzędziem, usprawniającym diagnozowanie HPVG i wspierającym podejmowanie decyzji klinicznych. Ze względu na zalety badania proponujemy je jako metodę obrazowania pierwszego wyboru w diagnostyce dzieci z HPVG.