Lactobacillus gasseri endocarditis on the aortic valve bioprosthesis - a case report.

Lactobacillus is a genus of Gram-positive, facultative anaerobic/ microaerophilic, rod-shaped bacteria. Lactobacilli constitute a significant component of the human microbiota in the oral cavity, gastrointestinal tract and female urogenital tract. They are also widely used as probiotics. Rarely, especially in patients with impaired immunity or with structural heart disease, do Lactobacilli become the pathogen responsible for serious infections, e.g. infective endocarditis (IE). CASE REPORT: The authors describe a case of an 80-year-old female with a past history of aortic valve replacement, mitral annuloplasty and pacemaker implantation admitted to hospital due to weakening, subfebrile state and chills. In transesophageal echocardiography vegetations on the aortic valve bioprosthesis were found, while pacemaker electrodes and mitral annulus were not involved in the endocarditis process. Bacteriological work-up revealed growth of L. gasseri. The patient was successfully treated with amoxicillin/clavulanate acid (six weeks) and with gentamycin (two weeks). No recurrence of the disease was observed during a six-month posthospital follow-up. Among different species of Lactobacillus, L. gasseri has not been reported as an IE pathogen so far. Lack of unequivocal data as to whether using probiotics may be responsible for infections, including IE, in patients with predisposing conditions.

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.

Shortening baroreflex delay in hypertrophic cardiomyopathy patients -- an unknown effect of ß-blockers.

AIMS: Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy and impaired diastolic and systolic function. Abnormal sympathetic-parasympathetic balance is a potential stimulus for left ventricular hypertrophy in HCM patients. ß-Blockers are routinely used in HCM for their strong negative inotropic effect; however, these drugs also influence the sympathetic-parasympathetic balance. This study aimed to determine the autonomic control of the cardiovascular system and the autonomic effects of ß-blockers in HCM patients treated or untreated with ß-blockers. METHODS: Among 51 HCM outpatients (18-70 years old; 29 men) there were 19 individuals with no medication and 32 subjects treated with a ß-blocker. Fourteen age- and gender-matched (23-70 years old; nine men) healthy volunteers were enrolled in the control group. Continuous, non-invasive finger blood pressure was recorded during supine rest for 30 min. Autonomic regulation of the cardiovascular system was measured by heart rate variability and spontaneous baroreflex function (cross-correlation sequence method). RESULTS: The mean pulse interval, time domain and spectral measures of heart rate variability and baroreflex sensitivity were comparable between HCM patients, treated or not with ß-blockers, and the control group. However, the delay of the baroreflex was significantly longer in HCM patients who were not treated with ß-blockers [2.0 (1.6-2.3) s] in comparison with HCM patients receiving ß-blockers [1.4 (1.1-1.8) s; P = 0.0072] or control subjects [1.2 (0.8-1.8) s; P = 0.0025]. This delay did not differ between HCM patients treated with ß-blockers and the control group. CONCLUSIONS: Hypertrophic cardiomyopathy not treated with ß-blockers is accompanied by prolonged baroreflex delay. The use of ß-blockers normalizes this delay.

Considerations for Home-Based Treatment of Fabry Disease in Poland during the COVID-19 Pandemic and Beyond.

Current therapy for Anderson-Fabry disease in Poland includes hospital or clinic-based intravenous enzyme replacement therapy with recombinant agalsidase alpha or beta, or oral pharmacological chaperone therapy with migalastat. Some countries around the world offer such treatment to patients in the comfort of their own homes. The 2020-2021 COVID-19 pandemic has pushed global healthcare providers to evolve their services so as to minimize the risk of COVID-19 exposure to both patients and providers; this has led to advances in telemedicine services and the increasing availability of at-home treatment for various procedures including parenteral drug administration. A total of 80% of surveyed Anderson-Fabry disease patients in Poland would prefer home-based treatment, which would be a safe and convenient alternative to clinic-based treatment if patient selection is based on our proposed algorithm. Our recommendations for home-based treatments appear feasible for the long term care of Anderson-Fabry disease patients during the COVID-19 pandemic and beyond. This may also serve as a basis for home-based treatment programs in other rare and ultra-rare genetic diseases.

Echocardiography during the coronavirus disease 2019 pandemic - the impact of the vaccination program. A 2021 update of the expert opinion of the Working Group on Echocardiography of the Polish Cardiac Society.

The coronavirus disease 2019 (COVID-19) pandemic resulted in an urgent need to reorganize the work of echocardiography laboratories in order to ensure the safety of patients and the protection of physicians, technicians, and other staff members. In the previous Expert Opinion of the Working Group on Echocardiography of Polish Cardiac Society we provided recommendations for the echocardiographic services, in order to ensure maximum possible safety and efficiency of imagers facing epidemic threat. Now, with much better knowledge and larger experience in treating COVID-19 patients and with introduction of vaccination programs, we present updated recommendations for performing transthoracic and transesophageal examinations, including information on the potential impact of personnel and the patient vaccination program, and growing numbers of convalescents on performance of echocardiographic laboratories, with the goal of their ultimate reopening.

Pol-CDRIE registry - 1-year observational data on patients hospitalized due to cardiac device-related infective endocarditis in Polish referential cardiology centres.

BACKGROUND: The rate of cardiac device-related infective endocarditis (CDRIE) is increasing worldwide, but no detailed data are available for Poland. AIMS: We aimed to evaluate clinical, diagnostic, and therapeutic data of patients hospitalized due to CDRIE in 22 Polish referential cardiology centers from May 1, 2016 to May 1, 2017. METHODS: Participating cardiology departments were asked to fill in a questionnaire that included data on the number of hospitalized patients, number and types of implanted cardiac electrotherapy devices, and number of infective endocarditis cases. We also collected clinical data and data regarding the management of patients with CDRIE. RESULTS: Overall, 99 621 hospitalizations were reported. Infective endocarditis unrelated to cardiac device was the cause of 596 admissions (0.6%), and CDRIE, of 195 (0.2%). Pacemaker was implanted in 91 patients with CDRIE (47%); cardioverter­defibrillator, in 51 (26%); cardiac resynchronization therapy­defibrillator, in 48 (25%); and cardiac resynchronization therapy­pacemaker, in 5 (2.5%). The most common symptoms were malaise (62%), fever/chills (61%), cough (21%), chest pain (19.5%), and inflammation of the device pocket (5.6%). Cultures were positive in 77.5% of patients. The cardiac device was removed in 91% of patients. The percutaneous approach was most common for cardiac device removal. All patients received antibiotic therapy, and 3 patients underwent a heart valve procedure. Transesophageal echocardiography was performed in 80% of patients. The most common complication was heart failure (25% of patients). CONCLUSIONS: The clinical profile, pathogen types, and management strategies in Polish patients with CDRIE are consistent with similar data from other European countries. Transesophageal echocardiography was performed less frequently than recommended. The removal rate in the Polish population is consistent with the general rates observed for interventional treatment in patients with CDRIE.

Sudden Cardiac Death in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium and is most often caused by mutations in sarcomere genes. The structural and functional abnormalities are not explained by flow-limiting coronary artery disease or loading conditions. The disease affects at least 0.2% of the population worldwide and is the most common cause of sudden cardiac death in young people and competitive athletes because of fatal ventricular arrhythmia. In some patients, however, HCM has a benign course. Therefore, it is of utmost importance to properly evaluate patients and single out those who would benefit from an implanted cardioverter defibrillator. In this article, we review and summarize the sudden cardiac death risk stratification algorithms, methods of preventing death due to HCM, and novel factors that may improve the existing prediction models.

Exercise testing in adults after repair of aortic coarctation: evaluation of cardiopulmonary exercise capacity and B-type natriuretic protein levels.

The aim of the study was to investigate, in adult patients after successful repair of aortic coarctation, potential relationships between B-type natriuretic peptide levels and exercise capacity and the following factors: arterial hypertension, residual stenosis of the ascending aorta, and age at the time of surgery. The study group comprised 74 patients (45 men) aged 19 to 61 years (mean, 31.2 +/- 9.8 yr), who had undergone surgery at the age of 0.5 to 34 years (mean, 10.4 +/- 6.8 yr). The surgery was performed between 5 and 34 years earlier (mean, 21.4 +/- 6.2 yr). A subgroup with residual aortic stenosis (significant when > or =25 mmHg) comprised 32 patients; a subgroup without residual stenosis comprised 42 patients. Patients were also divided into subgroups without arterial hypertension (n=32), with exercise-induced arterial hypertension (n=10), and with persistent arterial hypertension (n=32). All patients were in New York Heart Association functional class I. The control group comprised 30 healthy subjects (18 men) aged 26 to 46 years (mean, 32.2 +/- 6.6 yr). After testing exercise capacity in accordance with a modified Bruce protocol, we concluded that the exercise capacity of adults is reduced after surgical repair of aortic coarctation. This reduction is more pronounced in patients who have arterial hypertension, but it is unaffected by residual stenosis of the descending aorta. Serum B-natriuretic peptide concentrations, as determined by immunoradiometric assay, are significantly elevated, which may result from pressure overload of the left ventricle or from residual myocardial lesions due to coarctation repair at an older age.

Safety, feasibility and effectiveness of first in-human administration of muscle-derived stem/progenitor cells modified with connexin-43 gene for treatment of advanced chronic heart failure.

AIMS: To assess the safety and efficacy of transendocardial delivery of muscle-derived stem/progenitor cells with connexin-43 overexpression (Cx-43-MDS/PC) in advanced heart failure (HF). METHODS AND RESULTS: Thirteen subjects with advanced HF, New York Heart Association (NYHA) class II-III were enrolled and treated with targeted injection of Cx-43-MDS/PCs and then monitored for at least 6 months. Overexpression of Cx43 (Cx43+) was significantly higher in all but one subject (Cx43-). Injection of MDS/PCs was associated with significant improvement of exercise capacity: NYHA (3 ± 0 vs. 1.8 ± 0.7, P = 0.003), exercise duration (388.69 ± 141.83 s vs. 462.08 ± 176.69 s, P = 0.025), peak oxygen consumption (14.38 ± 3.97 vs. 15.83 ± 3.74 ml/kg.min, P = 0.022) and oxygen pulse (10.58 ± 2.89 vs. 18.88 ± 22.63 mLO2 /heart rate, P = 0.012). Levels of BNP, left ventricular (LV) ejection fraction and LV end-diastolic volumes tended to improve. There was a significant improvement of the mean unipolar voltage amplitudes measured for the injected segments and the entire left ventricle (9.62 ± 2.64 vs. 11.62 ± 3.50 mV, P = 0.014 and 8.83 ± 2.80 vs. 10.22 ± 3.41 mV, P = 0.041, respectively). No deaths were documented, Cx43+ (n = 12) subjects presented no significant ventricular arrhythmia; one Cx43- subject suffered from ventricular tachycardia (successfully treated with amiodarone). CONCLUSIONS: Injection of Cx-43-MDS/PCs in patients with severe HF led to significant improvement in exercise capacity and myocardial viability of the injected segments while inducing no significant ventricular arrhythmia. This may arise from improved electrical coupling of the injected cells and injured myocardium and thus better in-situ mechanical cooperation of both cell types. Therefore, further clinical studies with Cx43+ MDS/PCs are warranted.

The BNP concentrations and exercise capacity assessment with cardiopulmonary stress test in patients after surgical repair of Fallot's tetralogy.

Adult patients after surgical repair of Fallot's tetralogy usually present with satisfactory exercise capacity years after the original procedure, though they never tolerate as high levels of exercise as their healthy counterparts. The aim of the study was to assess exercise capacity with cardiopulmonary stress test and BNP levels in adult survivors of surgical repair of Fallot's tetralogy. We studied 60 patients with no or only mild symptoms (including 29 males), at the mean age of 27.6+/-8.2 years at the time of follow-up screening, operated on at the age of 7.5+/-5.3 years. In 34 patients moderate to severe pulmonary regurgitation (PR+) was observed. Control group consisted of 28 healthy volunteers (13 males), aged 28.7+/-5.1 years. Peak oxygen uptake (VO2) in studied group was found to be significantly lower than in control group (24.7+/-5.5 vs. 36.6+/-7.6 ml/kg/min, p=0.00001), VE/VCO2 slope, the marker of respiratory effort, was higher in surgical treatment group as compared to the control group (36.5+/-6.3 vs. 29.7+/-4.7, p=0.004). In patients with PR+, peak VO2% was higher than in PR- individuals (69.6+/-11.8% vs. 58.5+/-12.1%, p=0.0005). The BNP concentration in the studied group (34.8+/-27.1 pg/ml) was higher than in healthy subjects (11.5+/-6.5 pg/ml, p=0.00001). Levels of BNP correlated inversely with peak VO2 (r=-0.286, p=0.03), FVC (r=-0.265 p=0.04) and positively with VE/VCO2 (r=0.361, p=0.005). Additionally the levels of BNP correlated positively with the age of patients at the time of surgical repair (r=0.250, p=0.04). We concluded that exercise capacity in adults after repair of Fallot's tetralogy, especially those PR+, was lower than in healthy volunteers. Concentrations of BNP in surgical treatment survivors were higher and correlated well with cardiopulmonary stress study parameters.

Adults with Ebstein's anomaly--Cardiopulmonary exercise testing and BNP levels exercise capacity and BNP in adults with Ebstein's anomaly.

INTRODUCTION: Ebstein's anomaly is defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle. The aim of the study was to evaluate exercise capacity with cardiopulmonary stress testing and to determine plasma BNP levels in adults with Ebstein's anomaly, and to establish their relation with echocardiogaphic grading of the lesion severity. MATERIALS AND METHODS: Study group consisted of 21 patients (16 males, aged 40.3+/-11.5 years). The control group: 19 healthy individuals (13 males, aged mean 39.9+/-9.3 years). On echocardiography the grade of the lesion severity was calculated (EGE) and used to define the following four groups: I < 0.5, II: 0.5-0.9, III: 1.0-1.49, IV > 1.5. The forced vital capacity (FVC), first second forced expiratory volume (FEV1), peak oxygen uptake (peak VO2), and VE/VCO2 slope were assessed with cardiopulmonary stress test and plasma BNP levels measured with radioimmunometric assay. RESULTS: In the studied group VO2 was lower than in control (21.9+/-5.4 vs. 33.6+/-8.3 mL/kg/min [p = 0.00001]), VE/VCO2 slope was higher in Ebstein's group (40.1+/-8.1, p = 0.00001). BNP levels were higher in the Ebstein group then in controls (35.9+/-25.0 vs. 17.2+/-9.9 pg/mL [p = 0.0002]) and did not differ significantly between EGE groups. PeakVO2 of 24.5+/-3.9 in patients from II EGE group were higher than in patients from EGE groups: III (17.2+/-5.2 p = 0.007) and IV (22.9+/-4.7 p = 0.05). CONCLUSIONS: Exercise capacity of adults with Ebstein's anomaly is significantly reduced and plasma BNP levels are higher compared to healthy individuals. Exercise capacity in patients with Ebstein's anomaly becomes gradually lower alongside the EGE severity; however, BNP levels do not correlate significantly with this parameter.

[Cardiopulmonary exercise test in the evaluation of exercise capacity in patients with Ebstein anomaly and patent ostium secundum atrial septal defect aged over 30 years]. / Spiroergometryczna ocena wydolnosci wysilkowej u pacjentów po 30. roku zycia chorych na zespól Ebsteina oraz z droznym ubytkiem miedzyprzedsionkowym typu II.

UNLABELLED: There are few data available on applying the cardiopulmonary exercise test for evaluation of cardiac exercise capacity in adult patients with congenital heart diseases. The aim of the study was to perform this test in adults with Ebstein anomaly and patients with patent ASD II (atrial septal defect) and determination of potential relationships between these parameters and echocardiographically assessed hemodynamic indices. MATERIAL AND METHODS: Twenty patients with Ebstein anomaly mean aged 40.3 +/- 12.3 years were studied. Control group consisted of 19 individuals at mean age of 39.9 years. Echocardiography was performed for the evaluation of severity of the disease. Additionally, 36 patients with ASD II were included, mean age 44.7 +/- 8.2 years. The control group consisted of 25 individuals at mean age of 45.6 years. Maximum exercise treadmill test was carried out and resting and exercise spirometry. RESULTS: Ebstein anomaly. Maximum oxygen uptake, minute ventilation, maximum heart rate and blood pressure at peak exercise were significantly lower in study patients than in control group. VE/VCO2 was increased. No differences were observed between groups with respect to spirometric parameters. Oxygen uptake decreased along with the severity of the disease. ASD II. Maximum oxygen uptake, minute ventilation, maximum heart rate and blood pressure at peak exercise and spirometric parameters were significantly lower in study patients than in control group. Significant negative correlations were shown for VO2 (maximum oxygen uptake) and Qp:Qs (pulmonary to systemic flow ratio) (p = 0.004), maximum heart rate - HRmax and HRmax% and RV (end-diastolic right ventricular diameter) (p = 0.02 i p = 0.01), RV and systolic pressure at peak exercise (p = 0.03), obstruction marker FEV1 and RV (p = 0.04) and between RVSP (right ventricular end-systolic pressure) (p = 0.01). Negative correlation was observed between RQ (respiratory quotient) and RVSP (p = 0.004), and positive correlation between HRmax and VO2 (p = 0.005). CONCLUSIONS: The exercise capacity of adults with Ebstein anomaly and those with patent ASD II is significantly reduced. It decreases along with the echocardiographic severity of the disease in Ebstein anomaly patients and it seems to result from the right ventricular volume overload in patients with patent ASD II.

The new generation is coming. Percutaneous implantation of the fully repositionable Lotus® aortic valve prosthesis: the first Polish experience.

Transcatheter aortic valve implantation (TAVI) is nowadays an accepted method of treatment for patients with symptomatic severe aortic stenosis who are inoperable or at very high risk of classic surgical aortic valve replacement. The Lotus valve system is a new generation TAVI device composed of a self-expanding stent prosthesis with implemented bovine pericardial leaflets, which is designed to facilitate repositioning, resheathing, and retrieval, even in the fully expanded and functioning position before the final release. In addition, the Lotus valve is surrounded by a flexible membrane to seal paravalvular gaps between the prosthesis and native valve. We present the first Polish experiences with the Lotus valve system. Due to its unique features, the Lotus valve may improve the prognosis in patients with inoperable or high risk critical aortic stenosis.

Sustained improvement of clinical status and pulmonary hypertension in patients with severe heart failure treated with sildenafil.

BACKGROUND: Sildenafil, a phosphodiesterase-5 inhibitor, has been shown to decrease pulmonary vascular resistance (PVR) in patients with heart failure. The purpose of the study was to evaluate the effect of sildenafil on clinical status and pulmonary vascular reactivity in patients with congestive heart failure. MATERIAL AND METHODS: We enrolled 20 patients (18 men and 2 women, mean age 51 ± 12 years, diagnosed with congestive heart failure and pulmonary hypertension. This was a prospective, single-center study. Patients were treated with sildenafil 25 mg TDS for 12 months. Protocol included NYHA evaluation and repeated echocardiography, cardiac pulmonary stress tests, and right- sided catheterization. RESULTS: Initially, there were 16 (80%) patients in III NYHA status and 4 (20%) patients in II NYHA. After 12 months, 8 patients were in NYHA III (40%) status and 12 patients in NYHA II (60%). Peak oxygen consumption increased from 12 ± 3 ml/kg/min to 19 ± 4 ml/kg/min after 1-year therapy (p<0.001). The cardiac index increased from 3.1 ± 0.6 L/min/m2 to 3.6 ± 0.4 L/min/m2 (p<0.05). Pulmonary vasculature resistance decreased after 1-year therapy (4.7 ± 1 vs. 1.6 ± 0.5 Woods units (p<0.005) comparing to initials. Mean pulmonary artery pressure decreased to 23 ± 6 mmHg from 42 ± 5 mmHg (p<0.001) after 1-year therapy. CONCLUSIONS: One-year sildenafil therapy effectively improved clinical status and pulmonary vascular resistance in patients diagnosed with congestive heart failure.