Diagnosis and treatment of Fabry disease. Expert Opinion of the Polish Cardiac Society and the Polish Forum for Fabry Disease.

Fabry disease (FD) belongs to the group of lysosomal storage diseases (LSD), which are characterised by insufficient activity of enzymes responsible for the intra-lysosomal breakdown of various substrates. The result is an uncontrolled accumulation of by-products of cellular metabolism. Lysosomal storage diseases are inherited diseases, transmitted mainly in an autosomal recessive fashion.

Cardiac troponin I release after transcatheter closure of atrial septal defects is associated with supraventricular arrhythmias on early follow-up.

Introduction: Atrial septal defects (ASD) are prevalent congenital heart anomalies found in the adult population. Percutaneous ASD closure has become a routine clinical practice. Elevation of postprocedural transient cardiac biomarkers and exacerbation of supraventricular arrhythmias have been reported in the subject literature.

Tricuspid regurgitation: Frequency, clinical presentation, management and outcome among patients with severe left-sided valvular heart disease in Europe. Insights from the ESC-EORP Valvular Heart Disease II survey.

Aims: Tricuspid regurgitation (TR) is commonly observed in patients with severe left-sided valvular heart disease (VHD). This study sought to assess TR frequency, management and outcome in this population.

Methods And Results: Among 6883 patients with severe native left-sided VHD or previous left-sided valvular intervention enrolled in the EURObservational Research Programme prospective VHD II survey, moderate or severe TR was very frequent in patients with severe mitral VHD (30% when mitral stenosis, 36% when mitral regurgitation [MR]), especially in patients with secondary MR (46%), and rare in patients with severe aortic VHD (4% when aortic stenosis, 3% when aortic regurgitation).

Percutaneous aspiration of a right atrial thrombus with the AngioVac system.

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Infective endocarditis in adults with congenital heart disease: Contemporary management and related outcomes in Central and South-Eastern European region.

Background: Infective endocarditis (IE) remains major cause of morbidity and mortality in adult congenital heart disease (ACHD). Limited data exists on ACHD with IE in Central and South-Eastern European (CESEE) countries. The aim of this study is to characterize contemporary management and assess outcomes of ACHD with IE in CESEE region.

First two years of reimbursed enzyme replacement therapy in the treatment of Fabry disease in Poland.

Fabry disease (FD) is an ultra-rare genetic lysosomal storage disease caused by pathologic gene variants resulting in insufficient expression of α-galactosidase A. This enzyme deficiency leads to accumulation of globotriaosylceramide and globotriaosylsphingosine in plasma and in different cells throughout the body, causing major cardiovascular, renal, and nervous system complications. Until 2018, reimbursed enzyme replacement therapy (ERT) for FD was available in all European Union countries except Poland.

OBESITY AND ATRIAL FIBRILLATION - BARIATRIC SURGERY AS A METHOD OF AF RISK DECREASE.

The prevalence of obesity is growing at alarming rate worldwide. Obesity has reached the proportion of a global epidemic in both developed and developing countries. Growing number of obese patients with atrial fibrillation requires a closer look at how excessive body fat leads to AF.

PERSONALITY TRAITS OF PATIENTS SUFFERING FROM PFO AND ASD AND INFLUENCE OF COVID-19 PANDEMIC TIME FOR PATIENTS SUFFERING FROM CONGENITAL HEART DEFECTS.

Objective: Introduction: The work presents a research project carried out in John Paul II Hospital in Cracow in Clinical Department of Cardiac and Vascular Diseases with the Intensive Cardiac Supervision Subdivision, with participation of 100 (50 F, 50 M) patients with congenital heart defects. The purpose of the work is to resolve the issue of personality specifics, and thus the different characteristics of people who suffer from congenital heart defects. Therefore, the following questions should be answered: Is there a relationship between personality traits and the occurrence of a congenital heart defect? What personality traits are characteristic for patients with congenital heart defects? The aim: We aimed to assess personality traits of clients suffering from PFO and ASD.

Percutaneous closure of atrial septal defect: a consensus document of the joint group of experts from the Association of Cardiovascular Interventions and the Grown-Up Congenital Heart Disease Section of the Polish Cardiac Society.

Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications in adults are quite limited. This population has many unique characteristics and needs.

[The effectiveness of cardiovascular education in the prevention of heart disease among the inhabitants of Lesser Poland].

Objective: Introduction: Numerous risk factors for cardiovascular disease (CVD) are modifiable, therefore understanding their effects has a significant impact on lowering the incidence and mortality. The "Małopolska Cardiovascular Preventive Intervention Study (M-CARPI)" aims to educate the inhabitants of the voivodeship regarding preventative measures for developing CVD. The aim: To examine the effectiveness of the seminars regarding preventative measures for developing CVD according to the M-CAPRI among the inhabitants of Lesser Poland.

Psychological correlates of patients' identity suffering from atrial septal defect (ASD) and patent foramen ovale (PFO).

Background: The work presents a research project carried out in hospital with participation of 100 (50 female, 50 male) patients with congenital heart defects [atrial septal defect (ASD) and patent foramen ovale (PFO)]. The aim of the study was to identify specific personality traits of patients with congenital heart defects and to check the psychological functioning of patients by examining: the level of anxiety, impulsiveness, tendency to risk-taking, empathy, neuroticism, extraversion, psychoticism and lying. The presented results and their statistical analyses showed specific personality traits of patients with congenital heart defects.

Management of valvular and structural heart diseases during the coronavirus disease 2019 pandemic: an expert opinion of the Working Group on Valvular Heart Diseases, the Working Group on Cardiac Surgery, and the Association of Cardiovascular Interventions of the Polish Cardiac Society.

The ongoing pandemic of coronavirus disease 2019 (COVID‑19), caused by severe acute respiratory syndrome coronavirus 2 (SARS‑CoV‑2), represents a major challenge for healthcare. The involvement of cardiovascular system in COVID‑19 has been proven and increased healthcare system resources are redirected towards handling infected patients, which induces major changes in access to services and prioritization in the management of patients with chronic cardiovascular disease unrelated to COVID‑19. In this expert opinion, conceived by the task force involving the Working Groups on Valvular Heart Diseases and Cardiac Surgery as well as the Association of Cardiovascular Intervention of the Polish Cardiac Society, modification of diagnostic pathways, principles of healthcare personnel protection, and treatment guidelines regarding triage and prioritization are suggested.

Percutaneous left atrial appendage closure in a patient with atrial fibrillation and osler-weber-rendu disease.

We present a case report of a 79-year-old woman with permanent atrial fibrillation and Osler-Weber-Rendu disease who underwent percutaneous closure of left atrial appendage. The patient had a history of small bowel resection due to mesenteric embolism and recurrent episodes of gastrointestinal bleeding, epistaxis and hemarthrosis. Bleeding episodes were exacerbated by anticoagulation therapy causing severe anemia that required repeated red blood cells transfusions.

Personality traits of patients suffering from congenital heart defects.

Objective: Introduction: Personality traits of patients suffering from congenital heart defects The work presents a research project carried out in John Paul II Hospital, The Clinical Department of Cardiac and Vascular Diseases with the Intensive Cardiac Surgeon Division Institute of Cardiology, Collegium Medicum of the Jagiellonian University in Cracow, with participation of patients with congenital heart defects. We aimed to assess personality traits of clients suffering from congenital heart defects, in a group of women and men, younger, under 40 years old and older than 40 years old, with PFO and ASD before and after surgery. The aim: identify specific personality traits of patients with congenital heart defects and to check the psychological functioning of patients by examining: the level of anxiety, impulsiveness, tendency to risk-taking, empathy, neuroticism, extraversion, psychoticism and lying.

Interventional closure of patent foramen ovale in prevention of thromboembolic events. Consensus document of the Association of Cardiovascular Interventions and the Section of Grown‑up Congenital Heart Disease of the Polish Cardiac Society.

The presence of patent foramen ovale (PFO) was found to be associated with a number of medical conditions, including embolic stroke and recurrent transient neurological defects. The closure of PFO remains controversial; however, in recently published guidelines from the European Association of Percutaneous Cardiovascular Interventions in collaboration with 7 other European societies, which extensively refer to the latest randomized clinical trials, it is explicitly recommended to perform percutaneous PFO closure in the prevention of recurrent thromboembolic events. In connection with the above facts and expected increasing number of PFO closure procedures, the joint expert group of the Association of Cardiovascular Interventions and the Grown‑Up Congenital Heart Disease Section of the Polish Cardiac Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications, methods of performing procedures, and postoperative care in relation to Polish conditions and experiences.

[Elimination of the left atrial appendage in the prevention of thromboembolic events in patients with atrial fibrillation].

Atrial fibrillation (AF) is the most common cardiac arrhythmia in the adult population. Thromboembolic events are one of the reson of increased mortality in this group of patients. Oral anticoagulation therapy significantly reduces the risk of complications, however every tenth patient has contraindications to this treatment.

Transcatheter closure of atrial septal communication: Impact on quality of life in mid-term follow-up.

Background: Atrial septal defect (ASD) and patent foramen ovale (PFO) are specific types of atrial septal communications (ASC).

Objectives: We aimed to assess quality of life (QoL) in patients before and after percutaneous closure of ASC and determine the factors influencing QoL in this group of patients.

Material And Methods: We performed a clinical assessment and conducted an SF-36 questionnaire, electrocardiography and echocardiography studies in patients before and 6 months after percutaneous ASC closure.