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). An increase in TR grade was associated with a more severe clinical presentation and a poorer 6-month survival (p < 0.0001). Rates of concomitant tricuspid valve (TV) intervention at the time of left-sided heart valve surgery were high at the time of mitral valve surgery (50% when mitral stenosis, 41% when MR). Concordance between class I indications (patients with severe TR) for concomitant TV surgery at the time of left-sided valvular heart surgery according to guidelines and real-practice decision-making was very good (88% overall, 95% in patients operated on for MR). CONCLUSION: In this large international prospective survey among patients with severe left-sided VHD, moderate/severe TR was frequent in patients with mitral valve disease and was associated with a poorer outcome as TR grade increased. In patients with severe TR, compliance to guidelines for class I indications for concomitant TV surgery at the time of left-sided heart valve surgery was very good.

A review and recommendations for oral chaperone therapy in adult patients with Fabry disease.

Fabry disease (FD) is a rare, X-linked lysosomal storage disorder affecting both males and females caused by genetic abnormalities in the gene encoding the enzyme α-galactosidase A. FD-affected patients represent a highly variable clinical course with first symptoms already appearing in young age. The disease causes a progressive multiple organ dysfunction affecting mostly the heart, kidneys and nervous system, eventually leading to premature death. Disease-specific management of FD includes enzyme replacement therapy with agalsidase α and ß or pharmacological oral chaperone migalastat. Migalastat is a low-molecular-mass iminosugar, that reversibly binds to active site of amenable enzyme variants, stabilizing their molecular structure and improving trafficking to the lysosome. Migalastat was approved in the EU in 2016 and is an effective therapy in the estimated 35-50% of all patients with FD with amenable GLA gene variants. This position statement is the first comprehensive review in Central and Eastern Europe of the current role of migalastat in the treatment of FD. The statement provides an overview of the pharmacology of migalastat and summarizes the current evidence from the clinical trial program regarding the safety and efficacy of the drug and its effects on organs typically involved in FD. The position paper also includes a practical guide for clinicians on the optimal selection of patients with FD who will benefit from migalastat treatment, recommendations on the optimal selection of diagnostic tests and the use of tools to identify patients with amenable GLA mutations. Areas for future migalastat clinical research have also been identified.

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. METHODS: Data on ACHD patients with IE from 9 tertiary centres in 9 different CESEE countries between 2015 and 2020 was included. Baseline demographics, clinical presentation, indication for surgery, outcomes, hospital and all-cause-1-year mortality were studied. RESULTS: A total of 295 ACHD patients (mean age 40 ± 14 years) with IE were included. Median time from symptoms onset to establishing diagnosis was 25 (11-59) days. The majority of patients (203, 68.8%) received previous empiric oral antibiotic therapy. The highest incidence of IE was observed on native and left sided valves, 194(65.8%) and 204(69.2%), respectively. More than half had a vegetation size ≥10 mm (164, 55.6%); overall 138 (46.8%) had valve complications and 119 (40.3%) had heart failure. In-hospital mortality was 26 (8.8%). CONCLUSION: There is clear delay in establishing IE diagnosis amongst ACHD patients in CESEE countries. Adequate diagnosis is hampered by common prescription of empiric antibiotics before establishing formal diagnosis. Hence, patients commonly present with associated complications requiring surgery. Hospital treatment and survival are, nevertheless, comparable to other Western European countries. Improved awareness and education of patients and medical profession regarding IE preventive measures, risks, signs, and symptoms are urgently needed. Empiric antibiotic prescription before blood cultures are taken must be omitted.

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.             We present the preliminary results of the first two years of reimbursed ERT in Poland. We obtained data from the seven largest academic centers in Katowice, Kraków, Wroclaw, Poznan, Gdansk, Warszawa, and Lódz. The questionnaire included the following data: number of patients treated, number of patients qualified for ERT, and patient characteristics.             All centers returned completed questionnaires that included data for a total of 71 patients (28 men and 43 women) as of June 2021. Thirty-five patients with the diagnosis of FD confirmed by genetic testing (22 men and 13 women) had already qualified for reimbursed ERT. Mean (SD) age at the commencement of the ERT program was 39.6 (15.5) years (range 18-79 years). Mean time from the first clinical symptoms reported by the patients to the FD diagnosis was 21.1 (8.9) years, and the mean time from the final diagnosis of FD to the beginning of ERT was 4.7 (4.6) years.             FD is still underdiagnosed in Poland. To identify undiagnosed FD patients and to ensure that patients in Poland benefit fully from ERT, implementation of an effective nationwide screening strategy and close cooperation with a network of rare disease centers is advised.

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. Excessive adipose tissue is the source of many cardiovascular diseases, like hypertension, ischemic heart disease, and arrhythmias such as atrial fibrillation. The prevalence of atrial fibrillation in the general population is estimated at 1-2%. Obesity and overweight occur in 25% of patients with AF. Pathophysiology of obesity and the relationship between atrial fibrillation, diabetes, obstructive sleep apnea and metabolicsyndrome are presented in this article as well as the benefit of bariatric surgery in qualifying patients. Paroxysmal AF and its risk factors should be carefully assessed in all patients referred for bariatric surgery. Proper diagnosis allows physicians to introduce appropriate anticoagulant prophylaxis, and significantly lower complication rate. Antiarrhythmic, respiratory, and metabolic therapy should also be considered in preoperative and postoperative care.

European Society of Cardiology Working Group on Adult Congenital Heart Disease and Study Group for Adult Congenital Heart Care in Central and South Eastern European Countries consensus paper: current status, provision gaps and investment required.

AIMS: To examine the current status of care and needs of adult congenital heart disease (ACHD) services in the Central and South Eastern European (CESEE) region. METHODS AND RESULTS: We obtained data regarding the national ACHD status for 19 CESEE countries from their ACHD representative based on an extensive survey for 2017 and/or 2018. Thirteen countries reported at least one tertiary ACHD centre with a median year of centre establishment in 2007 (interquartile range 2002-2013). ACHD centres reported a median of 2114 patients under active follow-up with an annual cardiac catheter and surgical intervention volume of 49 and 40, respectively. The majority (90%) of catheter or surgical interventions were funded by government reimbursement schemes. However, all 19 countries had financial caps on a hospital level, leading to patient waiting lists and restrictions in the number of procedures that can be performed. The median number of ACHD specialists per country was 3. The majority of centres (75%) did not have ACHD specialist nurses. The six countries with no dedicated ACHD centres had lower Gross Domestic Product per capita compared to the remainder (P = 0.005). CONCLUSION: The majority of countries in CESEE now have established ACHD services with adequate infrastructure and a patient workload comparable to the rest of Europe, but important gaps still exist. ACHD care is challenged or compromised by limited financial resources, insufficient staffing levels, and reimbursement caps on essential procedures compared to Western Europe. Active advocacy and increased resources are required to address the inequalities of care across the continent.

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. The article also talks about how coronavirus pandemic affects patients with congenital heart disease. PATIENTS AND METHODS: Materials and methods: The research was conducted by psychologist Adrianna Skoczek. We performed a psychological clinical assessment and conducted the psychological tests like Eysenck Personality Questionnaire-Revised Short Version [EPQ-R(S)] and Eysenck's Impulsivity Inventory [IVE] by Hans J. Eysenck and Sybil G. Eysenck, the State-Trait Anxiety Inventory [STAI] by C. D. Spielberger, R. L. Gorsuch, R. E. Lushene describing personality traits of patients. Patients (F=50, M=50), with ASD (n=70) and with PFO (n=30). RESULTS: Results: In people suffering from ASD, the level of declared empathy was statistically significantly higher than the levels of impulsiveness and tendency to risk-taking. In people suffering from PFO, a statistically significant difference was observed only between the level of psychoticism and other variables. The level of declared empathy was statistically significantly higher than the levels of impulsiveness and tendency to risk-taking, a statistically significant difference was found between the analyzed variables - anxiety as a trait and anxiety as a condition. CONCLUSION: Conclusions: The results of the study allowed us to isolate the specific personality traits of patients suffering from congenital heart defects.

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. In recent years, significant progress has been made with regard to diagnostic modalities that facilitate the diagnostic workup of these patients. However, the decision­making process, especially in selected adult patients population (advanced age, various concomitant diseases, poor LV function [both systolic and diastolic], pulmonary hypertension, concomitant arrhythmias or multiple defects, or deficient rims) is still not easy. Available data are predominantly focused on imaging endpoints and short­term morbidity and mortality rather than long­­term. The evidence base for outcomes with or without defect closure comes from various studies with different observation periods. Moreover, the clinical experience in diagnosing and treating that subgroup of patients is inhomogeneous between individual physicians (cardiologists, imaging specialists, operators) and between small and large experience centers. In the view of the above, the joint group of experts from the Association of Cardiovascular Interventions and the Grown­Up Congenital Heart Disease Section of the Polish Cardiac Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications for treatment, methods of performing procedures, and tenets of postoperative care in Poland.

[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 "Malopolska 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. PATIENTS AND METHODS: Material and methods: The seminars were carried out by a doctor with a specialization in cardiology, in the form of presentations, based on materials prepared by experts in accordance with European and Polish guidelines. Both initially and after the course, participants completed questionnaires regarding the subjects discussed. RESULTS: Results: The study covered 558 people (137 men, 411 women). The analysis was based on the sub-populations of retirees and non-retirees. The knowledge of risk factors was checked, the respondents most often indicated incorrect judgment of nutrition, physical activity and smoking (45.9%, 39%, 44.2% for non-retirees and 22.8%, 27.6%, 16.5% for retirees, respectively). A significant increase in the number of people who indicated individual risk factors after the training was found (p=0,047 for HDL level and for others). CONCLUSION: Conclusions: Educating and creating awareness towards the prevention of CVD according to the M-CAPRI survey protocol is an effective tool for prevention. Due to the limited knowledge of cardiovascular risk factors, it is necessary to carry out activities to raise awareness of the importance of cardiological disease prevention. Preventive interventions are equally effective regardless of age or profession.

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. METHODS: The research was conducted by psychologist Adrianna Skoczek. We performed a psychological clinical assessment and conducted the psychological tests like Eysenck Personality Questionnaire-Revised Short Version [EPQ-R(S)] and Eysenck's Impulsivity Inventory (IVE) by Hans J. Eysenck and Sybil G. Eystenck, the State-Trait Anxiety Inventory (STAI) by C. D. Spielberger, R. L. Gorsuch, R. E. Lushene describing personality traits of patients. RESULTS: The level of extraversion was statistically significantly higher than the level of all other variables. The level of lies was significantly different from the level of psychoticism, which in turn was statistically significantly lower than all other. In the case of the examined women, statistically significant differences were found only between the level of psychoticism and all other analyzed variables. CONCLUSIONS: The conducted research shows that patients suffering from ASD or PFO have specific personality traits what allow to better understanding of suffering patients.

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 (COVID­19), caused by severe acute respiratory syndrome coronavirus 2 (SARS­CoV­2), represents a major challenge for healthcare. The involvement of cardiovascular system in COVID­19 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 COVID­19. 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. Heart Teams responsible for the treatment of valvular heart disease should continue their work using telemedicine and digital technology. Diagnostic tests must be simplified or deferred to minimize the number of potentially dangerous aerosol­generating procedures, such as transesophageal echocardiography or exercise imaging. The treatment of aortic stenosis and mitral regurgitation has to be offered particularly due to urgent indications and in patients with advanced disease and poor prognosis. Expert risk stratification is essential for triage and setting the priority lists. In each case, an appropriate level of personal protection must be ensured for the healthcare personnel to prevent spreading infection and preserve specialized manpower, who will supply the continuing need for handling serious chronic cardiovascular disease. Importantly, as soon as the local epidemic situation improves, efforts must be made to restore standard opportunities for elective treatment of valvular heart disease and occluder­based therapies according to existing guidelines, thus rebuilding the state ­of ­the ­art cardiovascular services.

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. PATIENTS AND METHODS: Material and methods: We performed a psychological clinical assessment and conducted the psychological tests like EPQ-R(S) by Hans J. Eysenck and Sybil G. Eysenck, IVE by Hans J. Eysenck and Sybil G. Eystenck, STAI by C. D. Spielberger, R. L. Gorsuch, R. E. Lushene describing personality traits of patients. RESULTS: Results: Patients (F=29, M=21), adult, with ASD and with PFO, with the level of education: basic, vocational, secondary, incomplete higher, higher; inhabiting: village, city up to 40 thousand residents, a city with a population of 41-61 thousand, and a city with a population of 60 thousand; civil status: single, married, divorced, widow/widower, separated; being: students, unemployed persons, working persons, pensioners, retirees. CONCLUSION: Conclusions: The presented results and their statistical analyses showed specific personality traits of patients with congenital heart defects.

Interventional closure of patent foramen ovale in prevention of thromboembolic events. Consensus document of the Association of Cardiovascular Interventions and the Section of Grown­up 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 Grown­Up 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. Percutaneous left atrial appendage occlusion (LAAO) is effective, non-pharmacological method of treatment through elimination the source of thromi. This procedure is dedicated to patients with high risk of thromboembolism events and contraindications to anticoagulation.

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. RESULTS: Patients with ASD (n = 56) had a lower SF-36 total score than those with PFO (n = 73), before and after percutaneous ASC occlusion (both p < 0.001). After the procedure, the improvement of SF-36 total score in patients with ASD or atrial fibrillation was greater (p < 0.001 and p = 0.005, respectively). We observed correlations between improvement of QoL and baseline supraventricular extrasystolic beats (rs = 0.28; p = 0.002), but not ventricular extrasystolic beats (rs = 0.03; p = 0.76). Quality of life improvement was predicted in patients with ASD by higher baseline tricuspid annular plane systolic excursion (TAPSE) and right ventricular longitudinal dimension R2 = 0.38; p < 0.001. However, in patients with PFO, this was predicted by TAPSE, lack of arterial hypertension and usage of angiotensin-converting enzyme inhibitors, R2 = 0.30; p < 0.001. CONCLUSIONS: Patients with ASD have lower QoL than those with PFO before and after percutaneous ASC occlusion. Six months after the procedure, the improvement of QoL in patients with ASD was higher than in those with PFO. The change in QoL self-assessed by patients after the procedure was associated with episodes of arrhythmia and was predicted with echocardiographic and clinical parameters.