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Fabry disease (FD) belongs to the group of lysosomal storage diseases (LSD), which are characterised by insufficient activity of enzymes responsible for the intra-lysosomal breakdown of various substrates. The result is an uncontrolled accumulation of by-products of cellular metabolism. Lysosomal storage diseases are inherited diseases, transmitted mainly in an autosomal recessive fashion. In the absence of a positive family history, an early diagnosis can often be missed. In addition, the age of clinical manifestation can range from infancy to adulthood - a distinction is made between severe "classic" variants of the disorders, presenting in childhood, and forms with late onset. Some of the conditions in this group may not show typical signs of tissue storage, such as liver and spleen enlargement, especially in subtypes associated with neurodegenerative changes. The aim of the Expert Opinion of the Polish Cardiac Society and the Polish Forum for Fabry Disease is to summarise the current knowledge on FD, present advances in diagnosis and therapy and disseminate known diagnostic and therapeutic algorithms for this group of patients.

Background: Silent cerebral ischemic lesions (SCILs) detected by magnetic resonance imaging (MRI) can precede symptomatic stroke, the risk of which is increased five-fold in atrial fibrillation (AF) patients. In our study, we aimed to evaluate the initial incidence of SCILs in the population of patients referred for ablation due to symptomatic AF and to identify possible risk factors., Methods: A total of 110 patients, with a mean age (SD) of 59.9 (9.4) years, referred for ablation, were included in the study. In all patients, MRI was performed before the procedure to evaluate the incidence of SCILs in the ablation-naïve patients., Results: MRI revealed preexisting SCIL in 81/110 patients (73.6%). Notably, SCILs were found in all patients with CHA₂DS₂-VASc score ≥ 4. In univariable analysis, age (p < 0.001), CHA₂DS₂-VASc score (p = 0.001), hypertension (p = 0.01), and anticoagulation duration (p = 0.023) were identified as significant risk factors for SCILs, while the presence of anatomical variants of left-sided common pulmonary veins trunk (LCPV) had negative prognostic value (p = 0.026). Multivariable logistic regression analysis identified age (p < 0.001) as the risk factor of preexisting SCILs, whereas the presence of LCPV trunk was associated with significantly lower (p = 0.005) SCILs incidence., Conclusions: Silent cerebral ischemic lesions detected in MRI are frequent in the population of patients with non-valvular AF. The incidence of SCILs is higher in patients with long history of arrhythmia and higher CHA₂DS₂-VASc score. The relationship between the anatomy of pulmonary veins and the incidence of SCILs needs further investigation.

Background: The use of subcutaneous implantable cardioverter-defibrillators (S-ICD) has been growing in Poland since 2014. The Polish Registry of S-ICD Implantations was run by the Heart Rhythm Section of the Polish Cardiac Society between May 2020 and September 2022 to monitor the implementation of that therapy in Poland., Aims: To investigate and present the state-of-the-art of S-ICD implantation in Poland., Methods: Implanting centers reported clinical data of patients undergoing S-ICD implantations and replacements, including age, sex, height, weight, underlying disease, history of pacemaker and defibrillator implantations, indications for S-ICD, electrocardiographical parameters, procedural techniques, and complications., Results: Four hundred and forty patients undergoing S-ICD implantation (411) or replacement (29) were reported by 16 centers. Most patients were in New York Heart Association class II (218 patients, 53%) or I (150 patients, 36.5%). Left ventricular ejection fraction was 10%-80%, median (IQR) was 33% (25%-55%). Primary prevention indications were present in 273 patients (66.4%). Non-ischemic cardiomyopathy was reported in 194 patients (47.2%). The main reason for the choice of S-ICD were: young age (309, 75.2%), risk of infectious complications (46, 11.2%), prior infective endocarditis (36, 8.8%), hemodialysis (23, 5.6%), and immunosuppressive therapy (7, 1.7%). Electrocardiographic screening was performed in 90% of patients. The rate of adverse events was low (1.7%). No surgical complications were observed.

Background: Cardiac implantable electronic device(CIED)infections are associated with significant morbidity, mortality, and increased healthcare expenses. Apart from standard systemic antibiotic therapy, locally acting agents are under investigation as a potential approach for the prevention of this complication., Aims: The study aimed to summarize our experience with a gentamycin-collagen sponge (GCS) in a multi-component prevention strategy of cardiac implantable electronic device infection., Methods: We retrospectively analyzed medical records of 312 consecutive patients who underwent CIED-related surgery and had at least a 6-month follow-up. All the individuals had GCS applied during surgery. An incidence of CIEDs-related infection in our group was compared to the risk level calculated according to the commonly used scores. Analysis of cost-effectiveness was also performed., Results: Incidence of CIED-related infection, defined as a primary endpoint, occurred relatively rarely (0.33%) as compared to the infection risk calculated according to commonly used scores Prevention of Arrythmia Device Infection Trial (PADIT) - 0.83%; CIED-AI - 0.90% or Mittal score - 1.00%; P<0.001 - for all). We did not record any complications related to GCS. We analyzed the cost-effectiveness of our GCS-based approach, which appeared to be financially beneficial (number needed to treat 149-200; difference of CIED infection treatment cost and GCSs price was 5093-26525 $)., Conclusions: We conclude that: (1) the use of GCS to reduce CIEDs infections is feasible and safe; (2) our multicomponent prevention strategy involving the GCS application seems to significantly reduce the rate of CIED infection, and it is cost-effective.

Background: Effectiveness of lesion formation during radiofrequency (RF) catheter ablation can be assessed using in vitro or in vivo animal models., Aims: In this in vitro study, we aimed to compare the prototypes of the first Polish RF ablation catheters with common commercially available catheters from other manufacturers., Methods: Samples of the porcine left ventricle were subject to temperature-controlled ablation (50 W / 50 oC / 60 s), using 4- and 8-mm -tip nonirrigated ablation catheters (commercial ones as well as new prototypes). The parameters of RF delivery were collected during energy applications. Subsequently, lesion dimensions were measured and compared between catheters., Results: Initial impedance and impedance drop during energy delivery differed significantly between catheters (both those with 4-mm and 8-mm tip electrodes). The maximum temperature was similar for 4-mm -tip catheters (P = 0.26), while it differed for 8-mm -tip ones (P <0.001). No significant differences between catheters were noted for lesion volume. The 8-mm -tip prototypes created lesions of greater depth (mean [SD], 5.8 [0.4] mm vs 4.7 [0.4] mm; P <0.001) and volume (mean [interquartile range], 239.4 [217.9-255.5] mm3 vs 173.7 [156.1-186.4] mm3; P <0.001) than those with a 4-mm tip electrode., Conclusions: Our study showed that RF ablation catheters with the same length of the tip electrode created lesions of similar volume, irrespective of the manufacturer and despite showing distinct physical parameters during energy applications. The Polish catheter prototypes showed similar performance as commercially available devices. Finally, 8-mm -tip prototypes produced lesions of greater depth and volume than those with a 4-mm tip electrode.

Background: Cryoablation is an effective and safe method of pulmonary vein isolation (PVI) for the treatment of atrial fibrillation (AF). However, monitoring of phrenic nerve function during cryoballoon PVI remains an important issue., Aims: We aimed to compare 2 techniques of phrenic nerve stimulation (PNS) with the use of either a straight or a crosier‑shaped decapolar deflectable catheter., Methods: The study included 218 patients (mean [SD] age, 61.8 [10.9] years; 87 women) referred for cryoballoon PVI for AF. Patients were randomly assigned to PNS with either a straight or a crosier‑shaped catheter., Results: The groups did not differ in demographic characteristics or PVI effectiveness. The current threshold during PNS was lower in the group in which the crosier‑shaped catheter was applied (mean [SD], 6.7 [4.9] mA vs 4.8 [3.7] mA; P <0.01). In this group, the procedure time was shorter (mean [SD], 72.6 [22.8] min vs 64.4 [14.8] min; P <0.01), fewer repositioning maneuvers were required (31.8% vs 19.4%; P <0.05), and atrial capture during PNS was observed more frequently (11.5% vs 29.6%; P <0.01)., Conclusions: Straight and crosier‑shaped catheter techniques are equally effective in monitoring for phrenic nerve palsy. The crosier‑shaped catheter maintains its position better, thus leading to fewer dislocations and requiring fewer correction maneuvers, which might correlate with shorter procedure time. Moreover, this technique provides lower pacing thresholds. Both techniques may be safely used in patients with AF, and if stable phrenic nerve capture cannot be achieved, switching to another technique seems reasonable.

We present a 46-year-old male patient with complete atrio-ventricular block. A inflammatory etiology was suspected and finally lyme carditis was diagnosed. The conduction abnormalities disappeared with antibiotic treatment and a pacemaker implantation was not needed. Further follow-up of two years was uneventful.

26-year old patient was admitted to our department with suspected Brugada syndrome (BrS). He complained of recurrent dizziness due to which he was taken by a team of rescuers to the district hospital, where the recorded ECG demonstrated isolated 3–7 mm ST segment elevation in leads V1–V3. Coronary angiography performed in this hospital was normal. Then patient was transferred to our department for consideration of ICD implantation. ECG registration in our hospital with 0.05 Hz (3.2 s) filter showed sinus bradycardia with 1–2 mm ST segment elevation in precordial leads. A detailed analysis of all ECG's proved that the district hospital ECG was performed in manual mode with the application of 1.5 Hz (0.1 s) low frequency high-pass filter. To confirm the effect of incorrect filter as the cause of misdiagnosis we repeated ECG recording using the recorder which allows to apply the same settings 1.5 Hz (0.1 s) as in district hospital and reconstructed ECG with repolarisation abnormalities. Negative ajmaline test (1 mg/kg) additionally reaffirmed an exclusion of dynamic form of BrS.

We present a case of a 36-year-old female patient with a long history of persistent palpitations, limited exercise tolerance, and pre-syncope. ECG recordings revealed long lasting episodes of inappropriate sinus tachycardia (IST). IST is characterised by persistent cardiac arrhythmias with sinus P waves in the ECG, and an exaggerated response to a minimal physical effort or stress. Using 24-h Holter definition the average heart rate in patients with IST should be > 90 bpm or daytime heart rate > 100 bpm. The syndrome is associated neither with structural heart disease nor with any reversible causes of sinus tachycardia (i.e. fever, anemia, infection, hyperthyroidism, drug abuse). Therefore the diagnosis is generally one of exclusion. Beta-blockers are most frequently used as first line therapy. Other potentially effective drugs are nondihydropyridine calcium antagonists or ivabradine. The prognosis in patients with IST is very good but in some cases the symptoms can be severe and debilitating.

Primary amyloidosis is a systemic disorder caused by the clonal production and tissue deposition of immunoglobulin light chain proteins. The disease symptoms are typical of multisystem failure. Common presenting features include nephrotic syndrome, hepatomegaly, sensomotor peripheral neuropathy and, in the case of cardiac involvement, congestive heart failure. This last sign appears very seldom as alone, without any others. Cardiac involvement generally denotes a poor prognosis, regardless of the method of treatment. The median survival rate from onset of congestive heart failure is 6 months. Only the patients with earliest diagnosis made and advanced treatment (chemotherapy, autologous stem-cell transplantation, heart transplantation) introduced have the chance of the lengthening of life. The authors present a case of 52-year-old man with a primary amyloidosis, who suffered from severe, not responding to treatment, congestive heart failure. Because of lack of the other organ involvement symptoms, the correct diagnosis was made very late. The authors place emphasis on a simple diagnostic tool such as the correlation between the low voltage in the limb ECG leads and the echocardiographic sings of left ventricular hypertrophy. The combination of specific ECG, echocardiographic findings and positive extracardiac tissue biopsy may be sufficient to reach correct diagnosis. These examinations are easy accessible in non-specialist hospitals.

Background: Acute myocardial infarction (AMI) is one of the main causes of death in the elderly, however, the optimal therapy of AMI in this age-group has not yet been established., Aim: To compare the early outcome of patients with AMI aged > or =75 years who underwent primary percutaneous coronary interventions (PCI) or were treated conservatively., Methods: In-hospital outcome of 180 patients aged > or =75 years, hospitalised due to AMI, was retrospectively analysed. Ninety two consecutive patients, admitted with AMI between May 2001 and October 2002, underwent primary PCI whereas 88 patients, treated for AMI between 1993 and 2002, received standard pharmacological therapy without thrombolysis., Conclusions: Primary PCI in the elderly with AMI is significantly more effective than conservative therapy, except with patients with cardiogenic shock in whom mortality is similar regardless of the therapy used.