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Background: Part of the current stereotactic arrythmia radioablation (STAR) workflow is transfer of findings from the electroanatomic mapping (EAM) to computed tomography (CT). Here, we analyzed inter- and intraobserver variation in a modified EAM-CT registration using automatic registration algorithms designed to yield higher robustness. Materials and methods: This work is based on data of 10 patients who had previously undergone STAR. Two observers participated in this study: (1) an electrophysiologist technician (cardiology) with substatial experience in EAM-CT merge, and (2) a clinical engineer (radiotherapy) with minimum experience with EAM-CT merge. EAM-CT merge consists of 3 main steps: segmentation of left ventricle from CT (CT LV), registration of the CT LV and EAM, clinical target volume (CTV) delineation from EAM specific points. Mean Hausdorff distance (MHD), Dice Similarity Coefficient (DSC) and absolute difference in Center of Gravity (CoG) were used to assess intra/interobserver variability. Results: Intraobserver variability: The mean DSC and MHD for 3 CT LVs altogether was 0.92 ± 0.01 and 1.49 ± 0.23 mm. The mean DSC and MHD for 3 CTVs altogether was 0,82 ± 0,06 and 0,71 ± 0,22 mm. Interobserver variability: Segmented CT LVs showed great similarity (mean DSC of 0,91 ± 0,01, MHD of 1,86 ± 0,47 mm). The mean DSC comparing CTVs from both observers was 0,81 ± 0,11 and MHD was 0,87 ± 0,45 mm. Conclusions: The high interobserver similarity of segmented LVs and delineated CTVs confirmed the robustness of the proposed method. Even an inexperienced user can perform a precise EAM-CT merge following workflow instructions.

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Background: Management of hypertrophic obstructive cardiomyopathy (HOCM) is often challenging, depending on clinical manifestation. This case report illustrates the complex treatment of HOCM with associated recurrent ventricular arrhythmias. Case summary: A 54-year-old female with HOCM diagnosed in 2012 underwent a failed attempt for alcohol septal ablation, implantation of an implantable cardioverter-defibrillator, and repeated radiofrequency ablations (including ablation of the septal bulge to reduce LV obstruction). For ventricular tachycardia (VT) recurrences, she had stereotactic arrhythmia radioablation with subsequent epicardial cryoablation from mini-thoracotomy, and endocardial ablation with pulsed field energy. The situation was finally solved by mechanical support and heart transplantation. Discussion: A few important lessons can be learned from the case. First, radiofrequency ablation was used successfully to decrease left outflow tract obstruction. Second, stereotactic radiotherapy has been used after four previous endo/epicardial catheter ablations to decrease the recurrences of VT. Third, mini-thoracotomy was used after previous epicardial ablation with subsequent adhesions to modify the epicardial substrate with cryoenergy. Fourth, pulsed field ablation of atrial fibrillation resulted in an excellent therapeutic effect. Fifth, pulsed field ablation was also used to modify the substrate for VT, and was complicated by transient AV block with haemodynamic deterioration requiring mechanical support. Finally, a heart transplant was the ultimate solution in the management of recurrent VT.

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AIMS: A pulsed electric field (PF) energy source is a novel potential option for catheter ablation of ventricular arrhythmias (VAs) as it can create deeper lesions, particularly in scarred tissue. However, very limited data exist on its efficacy and safety. This prospective observational study reports the initial experience with VA ablation using focal PF. METHODS AND RESULTS: The study population consisted of 44 patients (16 women, aged 61 ± 14years) with either frequent ventricular premature complexes (VPCs, 48%) or scar-related ventricular tachycardia (VT, 52%). Ablation was performed using an irrigated 4 mm tip catheter and a commercially available PF generator. On average, 16 ± 15 PF applications (25 A) were delivered per patient. Acute success was achieved in 84% of patients as assessed by elimination of VPC or reaching non-inducibility of VT. In three cases (7%), a transient conduction system block was observed during PF applications remotely from the septum. Root analysis revealed that this event was caused by current leakage from the proximal shaft electrodes in contact with the basal interventricular septum. Acute elimination of VPC was achieved in 81% patients and non-inducibility of VT in 83% patients. At the 3-month follow-up, persistent suppression of the VPC was confirmed on Holter monitoring in 81% patients. In the VT group, the mean follow-up was 116 ± 75 days and a total of 52% patients remained free of any VA. CONCLUSION: Pulsed electric field catheter ablation of a broad spectrum of VA is feasible with acute high efficacy; however, the short-term follow-up is less satisfactory for patients with scar-related VT.

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Catheter ablation (CA) has become an established treatment strategy for managing recurrent ventricular tachycardias (VTs) in patients with structural heart disease. In recent years, percutaneous mechanical circulatory support (PMCS) devices have been increasingly used intra-operatively to improve the ablation outcome. One indication would be rescue therapy for patients who develop haemodynamic deterioration during the ablation. However, more efforts are focused on identifying subjects who are at high risk of such deterioration and could benefit from the pre-emptive use of the PMCS. The third reason to use PMCS could be the inability to identify diffuse substrate, especially in non-ischaemic cardiomyopathy. This paper reviews available experiences using various types of PMCS in different clinical scenarios. Although PMCS allows mapping during VT, it does not significantly influence acute outcomes and not convincingly long-term outcomes. On the contrary, the complication rate appears to be higher in PMCS cohorts. Our data suggest that even in patients with severe left ventricular dysfunction, the substrate modification can be performed without the need for general anaesthesia and risk of haemodynamic decompensation. In end-stage heart failure associated with the electrical storm, implantation of a left ventricular assist device (or PMCS with a transition to the left ventricular assist device) might be the preferred strategy before CA. In high-risk patients who are not potential candidates for these treatment options, radiotherapy could be considered as a bail-out treatment of recurrent VTs. These approaches should be studied in prospective trials.

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AIMS: Patients with structural heart disease (SHD) undergoing catheter ablation (CA) for ventricular tachycardia (VT) are at considerable risk of periprocedural complications, including acute haemodynamic decompensation (AHD). The PAINESD score was proposed to predict the risk of AHD. The goal of this study was to validate the PAINESD score using the retrospective analysis of data from a large-volume heart centre. METHODS AND RESULTS: Patients who had their first radiofrequency CA for SHD-related VT between August 2006 and December 2020 were included in the study. Procedures were mainly performed under conscious sedation. Substrate mapping/ablation was performed primarily during spontaneous rhythm or right ventricular pacing. A purposely established institutional registry for complications of invasive procedures was used to collect all periprocedural complications that were subsequently adjudicated using the source medical records. Acute haemodynamic decompensation triggered by CA procedure was defined as intraprocedural or early post-procedural (<12 h) development of acute pulmonary oedema or refractory hypotension requiring urgent intervention. The study cohort consisted of 1124 patients (age, 63 ± 13 years; males, 87%; ischaemic cardiomyopathy, 67%; electrical storm, 25%; New York Heart Association Class, 2.0 ± 1.0; left ventricular ejection fraction, 34 ± 12%; diabetes mellitus, 31%; chronic obstructive pulmonary disease, 12%). Their PAINESD score was 11.4 ± 6.6 (median, 12; interquartile range, 6-17). Acute haemodynamic decompensation complicated the CA procedure in 13/1124 = 1.2% patients and was not predicted by PAINESD score with AHD rates of 0.3, 1.8, and 1.1% in subgroups by previously published PAINESD terciles (<9, 9-14, and >14). However, the PAINESD score strongly predicted mortality during the follow-up. CONCLUSION: Primarily substrate-based CA of SHD-related VT performed under conscious sedation is associated with a substantially lower rate of AHD than previously reported. The PAINESD score did not predict these events. The application of the PAINESD score to the selection of patients for pre-emptive mechanical circulatory support should be reconsidered.

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We present a case of a 32-year-old male with a history of palpitations and preexcitation on ECG who underwent altogether four failed catheter ablations using different approaches in the two other electrophysiology centers within two years. ECG showed overt preexcitation with a positive delta wave in lead I and negative in leads V1-V3, suggesting a right free wall accessory pathway. During the electrophysiological study, the accessory pathway was localized on the free lateral wall. However, the electrograms and mapping during atrial and ventricular pacing suggested the presence of true epicardial accessory pathway. Repeated radiofrequency energy delivery with the support of the steerable sheath and excellent contact (as assessed by intracardiac echocardiography) at the earliest ventricular activation was not successful. Therefore, the Farawave catheter (Boston Scientific, Inc) was used, and a flower configuration with the intention to cover the entire atrial attachment of the pathway during ventricular pacing was selected. Application of pulsed field resulted in interruption of accessory pathway conduction. An electrophysiological study one year later confirmed the persistent effect of ablation. This case illustrates the potential utility of pulsed field energy for the ablation of atrial insertion of the accessory pathway with an epicardial course. Such an approach can avoid epicardial mapping and access and may improve the safety of the procedure.

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BACKGROUND: Stereotactic arrhythmia radiotherapy (STAR) has been proposed recently in patients with refractory ventricular tachycardia (VT). OBJECTIVES: The purpose of this study was to describe the efficacy and safety of STAR in the Czech Republic. METHODS: VT patients were recruited in 2 expert centers after at least 1 previously failed catheter ablation (CA). A precise strategy of target volume determination and CA was used in 17 patients treated from December 2018 until June 2022 (EFFICACY cohort). This group, together with an earlier series of 19 patients with less-defined treatment strategies, composed the SAFETY cohort (n = 36). A dose of 25 Gy was delivered. RESULTS: In the EFFICACY cohort, the burden of implantable cardioverter-defibrillator therapies decreased, and this drop reached significance for direct current shocks (1.9 ± 3.2 vs 0.1 ± 0.2 per month; P = 0.03). Eight patients (47%) underwent repeated CA for recurrences of VT during 13.7 ± 11.6 months. In the SAFETY cohort (32 procedures, follow-up >6 months), 8 patients (25%) presented with a progression of mitral valve regurgitation, and 3 (9%) required intervention (median follow-up of 33.5 months). Two cases of esophagitis (6%) were seen with 1 death caused by the esophago-pericardial fistula (3%). A total of 18 patients (50%) died during the median follow-up of 26.9 months. CONCLUSIONS: Although STAR may not be very effective in preventing VT recurrences after failed CA in an expert center, it can still modify the arrhythmogenic substrate, and when used with additional CA, reduce the number of implantable cardioverter-defibrillator shocks. Potentially serious sides effects require close follow-up.

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Sudden cardiac death (SCD) might have an inherited cardiac condition background. Genetic testing supports post-mortem diagnosis and screening of relatives at risk. Our aim is to determine the feasibility of a Czech national collaboration group and to establish the clinical importance of molecular autopsy and family screening. From 2016 to 2021, we have evaluated 100 unrelated SCD cases (71.0% males, age: 33.3 (12.8) years). Genetic testing was performed by next-generation sequencing utilizing a panel of 100 genes related to inherited cardiac/aortic conditions and/or whole exome sequencing. According to autopsy, cases were divided into cardiomyopathies, sudden arrhythmic death syndrome, sudden unexplained death syndrome, and sudden aortic death. We identified pathogenic/likely pathogenic variants following ACMG/AMP recommendations in 22/100 (22.0%) of cases. Since poor DNA quality, we have performed indirect DNA testing in affected relatives or in healthy parents reaching a diagnostic genetic yield of 11/24 (45.8%) and 1/10 (10.0%), respectively. Cardiological and genetic screening disclose 83/301 (27.6%) relatives at risk of SCD. Genetic testing in affected relatives as starting material leads to a high diagnostic yield offering a valuable alternative when suitable material is not available. This is the first multidisciplinary/multicenter molecular autopsy study in the Czech Republic which supports the establishment of this type of diagnostic tests. A central coordinator and proper communication among centers are crucial for the success of a collaboration at a national level.

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Stereotactic body radiotherapy is a recent promising therapeutic alternative in cases of failed catheter ablation for recurrent ventricular tachycardias (VTs) in patients with structural heart disease. Initial clinical experience with a single radiation dose of 25 Gy shows reasonable efficacy in the reduction of VT recurrences with acceptable acute toxicity. Many unanswered questions remain, including unknown mechanism of action, variable time to effect, optimal method of substrate targeting, long-term safety, and definition of an optimal candidate for this treatment."

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BACKGROUND: Pulmonary vein isolation (PVI) by radiofrequency (RF) energy is associated with a collateral ganglionated plexi ablation. Pulsed electric field (PEF) is a nonthermal energy source that preferentially affects the myocardial cells and spares neural tissue. OBJECTIVES: This study investigated whether PVI by a PEF compared with RF energy will result in less prominent alteration of the cardiac autonomic nervous system. METHODS: A total of 31 patients with atrial fibrillation underwent PVI using a novel lattice-tip catheter and PEF energy (n = 18) or a conventional irrigated-tip catheter and RF energy (n = 13). The response of the sinoatrial node and atrioventricular node to extracardiac high-frequency, high-output, right vagal nerve stimulation was evaluated at baseline and during and at the end of the ablation procedure. Substantial reduction in responsiveness was arbitrarily defined as stimulation-inducible pause <1.5 seconds. RESULTS: Reduced response of the sinoatrial node was documented in 13 of 13 (100%) and 6 of 18 (33%) patients (P = 0.0001) in RF and PEF groups, respectively. Reduced response of the atrioventricular node was found in 10 of 11 (93%) and 6 of 18 (33%) patients (P = 0.002) in RF and PEF groups, respectively. The major effects were observed predominantly during ablation around the right pulmonary veins. Early recovery of ganglionated plexi function was noticed only in the PEF ablation group. RF ablation resulted in higher acceleration of the sinus rhythm compared with PEF ablation (20 ± 13 beats/min vs 12 ± 10 beats/min; P = 0.04). CONCLUSIONS: PEF compared with RF energy used for PVI induces significantly weaker and less durable suppression of cardiac autonomic regulations.

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Stereotactic body radiotherapy (SBRT) has been reported as an attractive option for cases of failed catheter ablation of ventricular tachycardia (VT) in structural heart disease. However, even this strategy can fail for various reasons. For the first time, this case series describes three re-do cases of SBRT which were indicated for three different reasons. The purpose in the first case was the inaccuracy of the determination of the treatment volume by indirect comparison of the electroanatomical map and CT scan. A newly developed strategy of co-registration of both images allowed precise targeting of the substrate. In this case, the second treatment volume overlapped by 60% with the first one. The second reason for the re-do of SBRT was an unusual character of the substrate-large cardiac fibroma associated with different morphologies of VT from two locations around the tumor. The planned treatment volumes did not overlap. The third reason for repeated SBRT was the large intramural substrate in the setting of advanced heart failure. The first treatment volume targeted arrhythmias originating in the basal inferoseptal region, while the second SBRT was focused on adjacent basal septum without significant overlapping. Our observations suggested that SBRT for VT could be safely repeated in case of later arrhythmia recurrences (i.e., after at least 6 weeks). No acute toxicity was observed and in two cases, no side effects were observed during 32 and 22 months, respectively. To avoid re-do SBRT due to inaccurate targeting, the precise and reproducible strategy of substrate identification and co-registration with CT image should be used.

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This case report describes a successful leadless pacemaker implant (Micra VR Medtronic, Inc, Minneapolis, MN) in a 48-year-old patient with a history of Mustard repair. Twenty-one years after dual-chamber pacemaker implant, both conventional leads became dysfunctional. Lead extraction was refused by the patient and the subclavian vein was obstructed. A leadless pacemaker was selected as an alternative. Intracardiac echocardiography allowed the safe introduction of the delivery system into the nonsystemic left ventricle. Four months after implant, the pacing parameters are stable and the patient is without new complaints. A leadless pacemaker could be considered in patients with complex grown-up congenital heart disease (GUCH).

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Intracardiac echocardiography (ICE) is the most practical method for online imaging during electrophysiological procedures. It allows guiding of complex catheter ablation procedures together with electroanatomical mapping systems, either with minimal or with zero fluoroscopy exposure. Besides safe and reproducible transseptal puncture, ICE helps to assess location and contact of the tip of the ablation catheter relative to specific anatomical structures. Another option is visualization of the arrhythmogenic substrate in patients with ventricular arrhythmias. This article describes the clinical utility of ICE in non-fluoroscopic electrophysiology procedures more in detail.

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AIMS: Catheter ablation (CA) for atrial fibrillation (AF) has a considerable risk of procedural complications. Major vascular complications (MVCs) appear to be the most frequent. This study investigated gender differences in MVCs in patients undergoing CA for AF in a high-volume tertiary center. METHODS: A total of 4734 CAs for AF (65% paroxysmal, 26% repeated procedures) were performed at our center between January 2006 and August 2018. Patients (71% males) aged 60 ± 10 years and had a body mass index of 29 ± 4 kg/m2 at the time of the procedure. Radiofrequency point-by-point ablation was employed in 96.3% of procedures with the use of three-dimensional navigation systems and facilitated by intracardiac echocardiography. Pulmonary vein isolation was mandatory; cavotricuspid isthmus and left atrial substrate ablation were performed in 22% and 38% procedures, respectively. MVCs were defined as those that resulted in permanent injury, required intervention, or prolonged hospitalization. Their rates and risk factors were compared between genders. RESULTS: A total of 112 (2.4%) MVCs were detected: 54/1512 (3.5%) in females and 58/3222 (1.8%) in males (p < .0001). On multivariate analysis, lower body height was the only risk factor for MVCs in females (p = .0005). On the contrary, advanced age was associated with MVCs in males (p = .006). CONCLUSION: Females have a higher risk of MVCs following CA for AF compared to males. This difference is driven by lower body size in females. Low body height in females and advanced age in males are independent predictors of MVCs. Ultrasound-guided venipuncture lowered the MVC rate in males.

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