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.

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.

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.

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.

[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.

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.

Clinical classification of rare cardiac arrhythmogenic and conduction disorders, and rare arrhythmias.

INTRODUCTION Rare cardiovascular diseases and disorders (RCDDs) constitute an important clinical problem, and their proper classification is crucial for expanding knowledge in the field of RCDDs. OBJECTIVES The aim of this paper is to provide an updated classification of rare arrhythmogenic and conduction disorders, and rare arrhythmias (RACDRAs). METHODS We performed a search for RACDRAs using the Orphanet inventory of rare diseases, which includes diseases with a prevalence of no more than 5 per 10 000 in the general population. We supplemented this with a search of PubMed and Scopus databases according to a wider definition proposed by the European Parliament and the Council of the European Union. RESULTS RACDRAs are categorized into 2 groups, primary electrical disorders of the heart and arrhythmias in specific clinical settings. The first group is further divided into subgroups of major clinical presentation: disorders predisposing to supraventricular tachyarrhythmias, ventricular tachyarrhythmias, bradyarrhythmias, and others. The second group includes iatrogenic arrhythmias or heart rhythm disturbances related to medical treatment, arrhythmias associated with metabolic disorders, and others. We provide a classification of RACDRAs and supplement them with respective RCDDs codes. CONCLUSION The clinical classification of RACDRAs may form a basis to facilitate research and progress in clinical practice, both in diagnostic and therapeutic approaches.

Transcatheter closure of atrial septal communication: impact on P-wave dispersion, duration, and arrhythmia in mid-term follow-up.

BACKGROUND: Atrial septal communications (ASCs) include atrial septal defects (ASDs) and patent foramen ovale (PFO). AIM: The purpose of this study was to assess P-wave dispersion (PWD) and the prevalence of arrhythmia in patients before and after ASC closure. METHODS: We analysed the clinical history and performed 12-lead electrocardiograms, echocardiograms, and 24-h Holter electrocardiograms in patients with ASC, before and six months after ASC closure. RESULTS: We included patients with ASD (n = 56) and PFO (n = 73). PWD before percutaneous ASC closure was predicted by right ventricular outflow tract (RVOT) proximal diameter, left atrial area, ASD, smoking, and paroxysmal dyspnoea, R2 = 0.67; p < 0.001. RVOT proximal diameter was an independent predictor of PWD, both in patients with ASD and PFO. Six months after successful closure of ASC, a reduction in PWD was observed in the whole group of patients as well as in patients with ASD and PFO considered separately. A decrease in PWD was associated with reduction of maximum P-wave duration. At the same time, in the whole group, we noticed a reduction in the number of supraventricular and ventricular extrasystolic beats and fewer atrial fibrillation (AF) episodes, p < 0.04 for all variables. Postprocedural AF episodes in patients with ASD were predicted by PWD of 80 ms. CONCLUSIONS: Percutaneous closure of ASC is associated with a reduction of PWD and fewer arrhythmia episodes six months after the procedure. PWD predicts AF episodes after ASD closure.

Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart.

BACKGROUND The Fontan procedure, performed for univentricular heart, may also include the technique of percutaneous fenestration to create a small atrial septal defect (ASD) and a right-to-left shunt. The aim of this study was to evaluate the long-term effects of fenestration in adult patients who had a Fontan procedure for univentricular heart. MATERIAL AND METHODS Fontan surgery was performed in 39 patients, including 19 (49%) patients with fenestration (Group I), and 20 (51%) patients without the fenestration procedure (Group II). Laboratory tests in both groups included echocardiography, plethysmography, cardiopulmonary exercise testing, and 24-hour Holter monitoring. RESULTS Compared with patients in Group I, patients in Group II had a significantly increased level of N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.04), alkaline phosphatase (ALP) (p=0.01) and a significant increase in frequency of atrial fibrillation (p=0.04). Patients in Group I had a significantly increased systemic ventricular ejection fraction (SVEF) (p=0.05) and increased heart rate (HR) (p=0.006), heart rate reserve (HRR) (p=0.02), ventilatory equivalent (VE) (p=0.01), and VO2 peak (p=0.05) on cardiopulmonary exercise testing (CPET). Renal, hematologic, and ventilatory parameters, and incidence of thromboembolism showed no significant differences between the groups. CONCLUSIONS Long-term follow-up of patients who underwent Fontan procedures with percutaneous fenestration had improved single ventricular function, lower NT-proBNP levels, improved exercise capacity, and reduced ALP levels. These findings indicate that percutaneous fenestration closure should be considered for adult patients who have undergone Fontan procedure for univentricular heart.

Aspirin resistance in adult patients after Fontan surgery.

BACKGROUND: Thrombotic complications are common in adult patients who have had a Fontan operation early in life for treatment of congenital heart disease. OBJECTIVE: To characterize platelet function and responsiveness to aspirin in relation to thrombogenesis, systemic inflammation, and markers of endothelial function in adults with Fontan circulation (FC). METHODS: Thirty-four FC patients (age 18-40years; 62% taking aspirin chronically and 38% not taking aspirin) and 32 age- and sex-matched healthy controls were studied. Platelet function was evaluated by measurement of basal concentrations of thromboxane B2 (TXB2) and sCD40L and ex-vivo generation of TXB2 and sCD40L. Plasma concentrations of thrombin-antithrombin, endothelin-1, vWF, IL-6, IL-8, MCP-1, MIP-1ß, TNFα, sVCAM-1, and syndecan-1 also were measured. RESULTS: Platelet numbers were significantly lower in FC patients than in controls, but the patients had significantly higher platelet activity, as evidenced by higher TXB2 and sCD40L concentrations and higher ex vivo generation of TXB2. Chronic aspirin treatment had no effect on plasma concentrations of TXB2 and sCD40L in FC, but in 52% of aspirin-treated FC subjects, TXB2 concentrations remained elevated at 60min of TXB2 generation, indicating aspirin resistance. In addition, FC patients had increased levels of thrombin-antithrombin, endothelin-1, vWF, IL-8, MCP-1, MIP-1ß, TNFα, sVCAM-1, and syndecan-1 but not of IL-6. CONCLUSION: Adults with FC had lower platelet numbers but increased platelet activity, increased thrombogenesis, systemic inflammation, and endothelial dysfunction. A significant proportion of aspirin-treated FC adults had aspirin resistance, which may be at least in part responsible for their increased incidence of thrombotic complications.

Transcranial Doppler ultrasonography should it be the first choice for persistent foramen ovale screening?

BACKGROUND: Persistent foramen ovale (PFO) is considered a cause of cryptogenic stroke and a risk factor for neurological events in young patients. The reference standard for identifying a PFO is contrast-enhanced transesophageal echocardiography (TEE).The goal of this study was to evaluate the feasibility of transcranial color Doppler (TCD) and its diagnostic sensitivity compared with TEE. METHODS: We investigated 420 patients admitted to our department with cryptogenic stroke, transient ischemic attacks or other neurological symptoms. All patients underwent TCD and TEE evaluation. TCD and TEE examinations were performed according to a standardized procedure: air-mixed saline was injected into the right antecubital vein three times, while the Doppler signal was recorded during the Valsalva maneuver. During TCD the passage of contrast into the right-middle cerebral artery was recorded 25 seconds following the Valsalva maneuver. RESULTS: We detected a right-to-left shunt in 220 patients (52.3%) and no-shunts in 159 patients (37.9%) with both TCD and TEE. In 20 (4.8%) patients TEE did not reveal contrast passage which was then detected by TCD. In 21 (5.0%) patients only TEE revealed a PFO. The feasibility of both methods was 100%. TCD had a sensitivity of 95% and a specificity of 92% in the diagnosis of PFO. CONCLUSIONS: TCD has a relatively good sensitivity and specificity. TCD and TEE are complementary diagnostic tests for PFO, but TCD should be recommended as the first choice for screening because of its simplicity, non-invasive character, low cost and high feasibility.

Arterial stiffness in adult patients after Fontan procedure.

OBJECTIVES: Increased arterial stiffness is a risk factor of atherosclerosis and cardio-vascular complications. The aim of the study was to determine whether peripheral vascular function might be an early marker of impaired health status in patients with a single ventricle after Fontan procedure. METHODS AND RESULTS: Twenty five consecutive adults (11 women and 14 men) aged 24.7 ± 6.2 years after the Fontan procedure and 25 sex, age and BMI match healthy volunteers underwent physical examination, blood analysis, transthoracic echocardiography and noninvasive assessment of aortic stiffness. Augmented pressure and Augmentation Index (AIx) were both significantly elevated in Fontan when compared to the controls (6,08 ± 0,7 vs. 2,0 ± 3,7; p = 0.002 and 17,01 ± 3,3 vs. 6,05 ± 11; p < 0.001, respectively). There were no differences in pulse wave velocity (PWV), mean blood pressure (BP), brachial pulse pressure (PP), central: systolic BP, diastolic BP and PP. In Fontan group we find negative correlation between PWV and SatO2 (r = -0.68; p = 0.04) and positive correlation with WBC (0.72; p = 0.72; p = 0.013), INR (0.81; p = 0.008), TNFα (r = 0.45; p = 0.04), and postoperative time (r = 0.77; p = 0.02). AIx correlates positively only with age at surgery (r = 0.45; p = 0.04). Bilirubin level correlates positively with brachial PP (r = 0.71; p = 0.02) and central PP (r = 0.68; p = 0.03).The multivariate model showed that SatO2 (ß = -0.44, p = 0.04) was the only independent predictor of PWV (R² = 0.32, p = 0.03). CONCLUSION: Adult Fontan patients have an increased arterial stiffness assessed by a noninvasive technique. Low arterial oxygen saturation postoperative time, age at surgery, white blood cells, TNFα and bilirubin level are associated with arterial stiffening in these patients. The combination of blood parameters of the hepatic function and noninvasive measurements of arterial stiffness could be helpful in comprehensive care of patients with Fontan circulation.

Relationship between carotid intima-media thickness, cytokines, atherosclerosis extent and a two-year cardiovascular risk in patients with arteriosclerosis.

AIM: To investigate the relationship between carotid intima-media thickness (CIMT), biomarkers, atherosclerosis extent and a two-year cardiovascular (CV) event risk in patients with arteriosclerosis. METHODS: The CIMT, levels of high-sensitivity C-reactive protein (hs-CRP), tumour necrosis factor alpha (TNF-α), transforming growth factor beta (TGF-ß), interleukin-6 (IL-6), interleukin-10 (IL-10), and NT-proBNP were measured in 279 subjects with atherosclerotic disease, mean age 64.1 ± 9.6 years. The patients were included when they had artery stenosis ≥ 50% in one, two, three or four arterial territories (coronary, supra-aortic, renal and/or lower limb arteries), and this was found in 97, 80, 69 and 33 patients, respectively. During a two-year follow-up, the incidences of CV death, myocardial infarction, ischaemic stroke and lesion progression were recorded. RESULTS: The identified independent predictors of ≥ 3-territorial stenoses ≥ 50% were CIMT > 1.3 mm (RR 1.72; p < 0.001), hs-CRP > 5 mg/dL (RR 1.28; p = 0.005), IL-6 > 6.5 pg/mL (RR 1.08; p = 0.089), IL-10 (RR 0.86; p = 0.002), diabetes (RR 1.11; p = 0.027), total-cholesterol (RR 1.21; p < 0.001), creatinine (RR 1.15; p = 0.004) and body mass index (RR 0.85; p = 0.001). During a two-year follow-up, CV events occurred in 52 (18.6%) patients. The CIMT > 1.3 mm (p < 0.001), diabetes (p = 0.018), TNF-α > 6 pg/mL (p = 0.018), LDL-cholesterol > 3.35 mmol/L (p = 0.012) and NT-proBNP (p = 0.074) were independent CV event risk factors associated with a 27%, 14%, 15%, 15% and 11% higher CV risk, respectively. However, after adjustment for a baseline location of artery stenosis ≥ 50%, CIMT became a non-significant predictor (p = 0.245). CONCLUSIONS: Levels of hs-CRP, IL-6, IL-10 are independently associated with atherosclerosis extent, while TNF-α and NT- -proBNP are mostly related to a two-year CV event risk. The CIMT > 1.3 mm seems to be a clinically relevant marker associated with atherosclerosis extent and CV risk, although CV event risk is primarily related to the baseline stenosis location.