Importance of Comprehensive Assessment in Brugada Syndrome. Reply to Kataoka, N.; Imamura, T. How to Diagnose and Risk Stratify Brugada Syndrome. Comment on "Matusik et al. Twelve-Lead ECG, Holter Monitoring Parameters, and Genetic Testing in Brugada Syndrome: Insights from Analysis of Multigenerational Family with a History of Sudden Cardiac Arrest during Physical Activity. J. Clin. Med. 2023, 12, 6581".

We would like to thank Dr. Imamura for their interest in our study and their valuable comments on diagnostics and risk stratification in Brugada syndrome (BrS) [...].

Twelve-Lead ECG, Holter Monitoring Parameters, and Genetic Testing in Brugada Syndrome: Insights from Analysis of Multigenerational Family with a History of Sudden Cardiac Arrest during Physical Activity.

Brugada syndrome (BrS) is an arrhythmogenic disorder increasing the risk of syncopal episodes and sudden cardiac death. BrS usually runs through families with reduced penetrance and variable expression. We analyzed the multigenerational family of a patient who died after sudden cardiac arrest with post-mortem diagnosis of BrS. We analyzed clinical history, comprehensive arrhythmic risk, genetic findings, and additional tests, including electrocardiogram (ECG), detailed 24-hour Holter ECG results, and standard echocardiography findings, and followed up the patients in the ambulatory clinic. We analyzed a pedigree of 33 members of four generations of the family (19 male and 14 female patients). In this family, we identified 7 patients with BrS (median Modified Shanghai Score and Sieira model: 4.5 (4-6) and 1 (0-4) points, respectively), including both parents of the deceased patient, and 8 relatives with negative sodium channel blocker drug challenge test. Genetic testing revealed a novel mutation in sodium voltage-gated channel alpha subunit 5 (SCN5A) c.941A>G, (p.Tyr314Cys) inherited from the father of the proband. Patients with BrS were characterized by longer P-wave duration (120 (102-155) vs. 92.5 (88-110) ms, p = 0.013) and longer PR intervals (211.3 ±26.3 vs. 161.6 ± 18.9 ms, p = 0.001), along with more frequent positive aVR sign, but did not differ in terms of QRS duration or T-wave characteristics in resting ECGs. BrS patients were characterized by lower mean, minimal, and maximal (for all p ≤ 0.01) heart rates obtained from Holter ECG monitoring, while there was no difference in arrhythmias among investigated patients. Moreover, visual diurnal variability of ST segment changes and fragmented QRS complexes were observed in patients with BrS in Holter ECG monitoring. There were no major arrhythmic events during median follow-up of 68.7 months of alive BrS patients. These results suggest ECG features which may be associated with a diagnosis of BrS and indicate a novel SCN5A variant in BrS patients. Twelve-lead Holter ECG monitoring, with modified precordial leads placement, may be useful in BrS diagnostics and risk stratification in personalized medicine.

Increased Levels of Platelets and Endothelial-Derived Microparticles in Patients With Non-Valvular Atrial Fibrillation During Rivaroxaban Therapy.

It is known that atrial fibrillation (AF) is associated with the procoagulant state. Several studies have reported an increase of circulating microparticles in AF, which may be linked to a hypercoagulable state, atrial thrombosis and thromboembolism. We evaluated in our study alterations in both platelet (PMP, CD42b) and endothelial-derived (EMP, CD144) microparticle levels on anticoagulant therapy with rivaroxaban in nonvalvular AF. After administration of rivaroxaban, PMP levels were increased (median, [IQR] 35.7 [28.8-47.3] vs. 48.4 [30.9-82.8] cells/µL; P = 0.012), along with an increase in EMP levels (14.6 [10.0-18.6] vs. 18.3 [12.9-37.1] cells/µL, P < 0.001). In the multivariable regression analysis, the independent predictor of post-dose change in PMPs was statin therapy (HR -0.43; 95% CI -0.75,-0.10, P = 0.011). The post-dose change in EMPs was also predicted by statin therapy (HR -0.34; 95% CI -0.69, -0.01, P = 0.046). This study showed an increase in both EMPs and PMPs at the peak plasma concentration of rivaroxaban. Statins have promising potential in the prevention of rivaroxaban-related PMP and EMP release. The pro-thrombotic role of PMPs and EMPs during rivaroxaban therapy requires further study.

Flow Cytometric Assessment of Endothelial and Platelet Microparticles in Patients With Atrial Fibrillation Treated With Dabigatran.

The prothrombotic state in patients with atrial fibrillation (AF) is related to endothelial injury, the activation of platelets and the coagulation cascade. We evaluated the levels of platelet- (CD42b) and endothelial-derived (CD144) microparticles in the plasma patients with non-valvular AF treated with dabigatran at the time of expected minimum and maximum drug plasma concentrations. Following that, we determined the peak dabigatran plasma concentration (cpeak ). CD42b increased after taking dabigatran (median [IQR] 36.7 [29.4-53.3] vs. 45.6 [32.3-59.5] cells/µL; p = 0.025). The concentration of dabigatran correlated negatively with the post-dabigatran change in CD42b (ΔCD42b, r = -0.47, p = 0.021). In the multivariate model, the independent predictors of ΔCD42b were: cpeak (HR -0.55; with a 95% confidence interval, CI [-0.93, -0.16]; p = 0.007), coronary artery disease (CAD) (HR -0.41; 95% CI [-0.79, -0.02]; p = 0.037) and peripheral artery disease (PAD) (HR 0.42; 95% CI [0.07, 0.74]; p = 0.019). CD144 did not increase after dabigatran administration. These data suggest that low concentrations of dabigatran may be associated with platelet activation. PAD and CAD have distinct effects on CD42b levels during dabigatran treatment.

Assessment of Left Atrial Function in Patients with Paroxysmal, Persistent, and Permanent Atrial Fibrillation using Two-Dimensional Strain.

BACKGROUND AND PURPOSE: Atrial fibrillation (AF) has a progressive nature, leading to structural, functional, and electrical changes in the left atrium (LA). Enhanced response to treatment in patients with AF can be achieved through improved knowledge of atrial structure and a better understanding of its function. The aim of this study was to assess LA strain and its determinants in patients with paroxysmal (PAF), persistent (PsAF), and permanent AF (PmAF). METHODS: Fifty-eight patients with registered non-valvular AF were divided into 3 groups depending on the type of AF. The participants underwent transthoracic echocardiography to assess the anatomy and function of heart chambers. Left atrial longitudinal strain (LALS) was measured in four-chamber projections using two-dimensional speckle tracking echocardiography. RESULTS: Patients with PAF had higher LALS (15.7±12.0) when compared to those with PsAF (4.3±7.9) and PmAF (5.8±7.8, all P=0.003). Multiple linear regression showed that the independent predictors of LALS were diastolic blood pressure (ß=0.95, R2=0.88) in the PAF group; left atrial area (ß=-0.56) and creatinine (ß=-0.63, R2=0.58) in the PsAF group; AF duration (ß=0.89) in the PmAF group (R2=0.72). CONCLUSION: LA strain has different determinants depending on AF type. LA size, renal function, and AF duration determine LALS in long-lasting AF. LA strain is a simple and accurate technique to estimate LA dysfunction in patients with long-lasting AF.

[Type and frequency of cardiac symptoms in patients with pulmonary sarcoidosis]. / Rodzaj i czestosc wystepowania objawów kardiologicznych u chorych na sarkoidoze pluc.

AIM: The aim of the study was to assess the type and frequency of prevalence of cardiac symptoms in patients with pulmonary sarcoidosis. MATERIALS AND METHODS: The study group consisted of 54 patients (21 female, 33 male), with biopsy-proven pulmonary sarcoidosis. Mean age was 45.85 +/-11.77 years. According to time passed from diagnosis of sarcoidosis patients were divided into 2 subgroups. Additionally, age, sex, left ventricular ejection fraction, cigarette smoking and comorbidities were analyzed. Course and stage of sarcoidosis were also included in the analysis. RESULTS: The frequency of cardiovascular symptoms in the study group was 87.04%. 59.26% of patients reported chest pain, the same subjects reported dyspnoea. 48.15% of respondents reported heart palpitations, 33.33% pre-syncope states, 12.96% syncope, and 37.04% edema of lower limbs. There were no statistically significant differences in the incidence of analyzed symptoms, depending on the duration of lung sarcoidosis. CONCLUSIONS: Patients with pulmonary sarcoidosis who report cardiovascular symptoms require further diagnostics.

[Evaluation of the clinical suitability of automated left ventricle's fraction and volume measurements in 3-dimensional echocardiography compared to values obtained in magnetic resonance imaging (pilot study)]. / Ocena przydatnosci klinicznej automatycznego pomiaru frakcji i objetosci lewej komory w echokardiografii przezklatkowej 3D w porównaniu do wartosci uzyskanych w rezonansie magnetycznym (badanie pilotazowe).

Recently a lot of authors have been trying to determine the usefulness of 3-dimensional echocardiography (TTE 3D) in evaluation of ejection fraction and left ventricular volume, but few attempt to compare it to the current gold standard of cardiac magnetic resonance (CMR). 3D imaging technics allows to avoid errors caused by geometry of the heart chambers and foreshortened views. American Echocardiographic Guidelines recommend the use of 3-dimensional echocardiography in daily clinical practice. AIM: The aim of the study was to establish clinical usefulness of automated 3D TTE software to calculate left ventricle ejection fraction (LVEF), left ventricle end diastolic volume (LVEDV) and left ventricle end systolic volume (LVESV), and to compare those measurements calculated in CMR and in 3D TTE. MATERIALS AND METHODS: The aim of the study was to establish clinical usefulness of automated 3D TTE software to calculate left ventricle ejection fraction (LVEF), left ventricle end diastolic volume (LVEDV) and left ventricle end systolic volume (LVESV), and to compare those measurements calculated in CMR and in 3D TTE. RESULTS: The mean LVEF in 3D TTE was 65% +/- 12%; LVEDV 123 ml +/- 67 ml, LVESV 42 ml +/- 29 ml. The CMR LVEF in the study group was 61% +/- 9%, LVEDV 134 ml +/- 51ml, LVESV 54 ml +/- 33 ml. Wilcoxon rank test showed no difference between medians of the measurements, the correlation coefficient between LVEF in 3D TTE and CMR was R = 0.84 (p = 0.036). LVEF calculated in 3D TTE shows good correlation with LVEF computed in CMR. However good visualization of the endocardium, especially in the apex, is essential. The volume of left ventricle is underestimated in 3D TTE. In previous studies underestimation of LVEDV and LVESV was explained by exclusion of endocardial trabeculae from the left ventricle cavity in automated measurement. CONCLUSIONS: The automated 3D TTE software allows simple, fast and precise evaluation of parameters of the left ventricle - especially LVEF. Automated 3D TTE software gives hope for the inclusion of 3D TTE in routine clinical practice due to its repeatability and easy use of the Heart Model software.

[Exercise capacity in patients with pulmonary sarcoidosis]. / Wydolnosc fizyczna u chorych na sarkoidoze pluc.

Exertional dyspnea is a common manifestation of sarcoidosis. Cardiopulmonary exercise testing (CPET) is a useful tool to evaluate exercise tolerance of sarcoid patients. AIM: The aim of the study was to evaluate of exercise capacity in patients with pulmonary sarcoidosis with regard to duration of the disease. Analysis of differences in physical tolerance between patients with cardiac sarcoidosis and without cardiac sarcoidosis. MATERIALS AND METHODS: 39 patients diagnosed with pulmonary sarcoidosis were enrolled to our study. Cardiopulmonary exercise test was used to assess exercise capacity. According to time passed from diagnosis of sarcoidosis patients were grouped into 2 groups. Cardiac sarcoidosis was diagnosed in 9 subjects (23.07%). The control group consisted of 33 healthy volunteers. RESULTS: The results of the ergospirometry test in patients with lung sarcoidosis showed statistically significant differences in comparison to the control group. The age of patients with pulmonary sarcoidosis correlated negatively with maximal heart rate, oxygen consumption at peak exercise and at anaerobic threshold, breathing frequency at peak exercise, minute ventilation at peak exercise and metabolic equivalent. There was a negative, statistically significant correlation between the oxygen pulse at the peak of exercise and the treatment of steroids in the past. CONCLUSIONS: Exercise limitation in patients with pulmonary sarcoidosis is a consequence of both ventilatory and cardiocirculatory impairment. Patients with longer disease history of sarcoidosis achieved worse results at cardiopulmonary exercise test than patients with shorter disease history. Trend towards worse exercise tolerance in patients with cardiac sarcoidosis compared to patients without cardiac sarcoidosis was observed.

[Lyme carditis]. / Borelioza serca.

Lyme disease is a multisystem infectious disease caused by the spirochete Borrelia burgdorferi. A steady increase in the number of cases is noticed both in Poland and Europe. Cardiac involvement in the course of borreliosis is relatively rare. It is estimated that it concerns about 0.5-10% of patients with Lyme disease. Cardiac involvement generally occurs in the early phase of illness. The most common manifestation of Lyme carditis are transient conduction abnormality, arrhythmias, myocarditis and pericarditis. The basic method of treatment Lyme carditis are antibiotics. The clinical course is usually benign. In most cases a complete recovery is observed. However, in a small proportion of patients dilated cardiomyopathy may occur. Furthermore, death from Lyme carditis has been reported. Lyme carditis remains a real diagnostic and therapeutic challenge for clinicians. Factors that can make the diagnosis difficult are: atypical clinical picture, negation of tick bite, the absence of erythema migrans, onset of symptoms outside the period of tick activity and negative serological results in the initial stage of the disease.

Assessment of left ventricle function in aortic stenosis: mitral annular plane systolic excursion is not inferior to speckle tracking echocardiography derived global longitudinal peak strain.

BACKGROUND: Early detection of left ventricle (LV) systolic dysfunction is essential for management of patients with aortic stenosis (AS). Two- dimensional speckle tracking derived global longitudinal peak strain (GLPS) is more sensitive than ejection fraction (EF) but requires good image quality and is not easily accessible. The aim of the study was to compare GLPS with traditional echocardiographic parameter- mitral annular plane systolic excursion (MAPSE) in AS. MATERIAL AND METHODS: In consecutive patients with moderate to severe AS and LV ejection fraction ≥ 50% standard echocardiography and two-dimensional speckle tracking echocardiography were performed. Mitral annular plane systolic excursion and global longitudinal peak strain were obtained from apical echocardiographic views. RESULTS: A total of 82 patients were examined, median age was 68 (60-78), 56% of them were men. There was a positive correlation between aortic valve area index (AVAI) and: MAPSE (r = 0.334, p = 0.002), MAPSE indexed for body surface area- MAPSEI (r = 0.349, p = 0.001) and GLPS (r = 0.342, p = 0.002) but not EF (r = 0.031, p = 0.782). A positive correlation was found between GLPS and MAPSE (r = 0.558, p < 0.001) and between GLPS and MAPSEI (r = 0.543, p < 0.001). All above parameters were significantly lower in symptomatic patients compared to asymptomatic subjects (GLPS: -13.82 ± 3.56 vs. -16.39 ± 3.16%, p = 0.002, MAPSE: 10.49 ± 1.91 vs. 11.95 ± 1.82 mm, p = 0.001 and MAPSEI: 5.66 (4.83-6.6) vs. 6.46 ± 0.97 mm/m2, p = 0.005). CONCLUSION: Despite the development of the modern echocardiographic techniques, mitral annular plane systolic excursion can still be used as a sensitive tool to detect early longitudinal LV systolic dysfunction.

[Vasodilatator testing with nitric oxide (bronchial control treatment system--BCTS) in patients with pulmonary hypertension]. / Testy wziewnego, sterowanego oddychaniem podawania tlenku azotu u chorych z nadcisnieniem plucnym.

Nitric oxide (NO) is one of the most important mediators produced in the human organism. It participates in the regulation of blood vessel lumens, activation of leucocytes and platelet; it is a mediator in the nervous system and in inflammation reactions. It was proved that in cases of patients with pulmonary hypertension, a decreased secretion of nitric oxide and an increased synthesis of endothelin-1 is observed. Therefore, in case of patients with pulmonary hypertension the exogenous, inhaled nitric oxide (iNO) is applied. It is added to the respiratory mixture and it passes through the alveolar-capillary barrier to the smooth muscle cells where it activates a guanyle cyclase, similarly to the physiologically produced nitric oxide. It was proved that it decreases pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP). Inhaled nitric oxide is applied for treatment purposes to patients after cardiosurgical operations, mainly heart transplantation and correction of valvular defects with accompanying pulmonary hypertension, as well as after implantation of the left ventricular assist device in order to relieve the right chamber. In case of patients qualified for cardiosurgical operations with the accompanying pulmonary hypertension as well as in case of patients with the arterial pulmonary hypertension a diagnostical test using iNO is carried out in order to determine further course of therapeutical treatment. The application of the new method of iNO administration by the BCTS (Bronchial Control Treatment System) method allows for a precise administration of accurately determined doses of iNO and its full utilisation through addition to the respiratory mixture in the initial phase of inspiration. The risk of side effects is also decreased; so far no influence on the circulatory system or an increase of the level of methemoglobin was observed.