AIMS: Stereotactic arrhythmia radioablation (STAR) has emerged as bail-out treatment for ventricular tachycardia (VT). Accurate, reproducible, and easy-to-use data transfer from electroanatomical mapping (EAM) systems to radiotherapy planning CT is desirable. We aim to evaluate interobserver variability, ease of use, and learning curve for EAM based target volume (CardTV-EPinv) creation and transfer using available software packages. METHODS AND RESULTS: In patients considered for STAR, CardTV-EPinv were created using ADAS and Slicer3D for workflow comparison. Four CardTV-EPinv (clinically targeted volume and three mock targets) were created by an experienced operator and a 2nd-year medical student, based on endocardial EAM tags indicating VT substrate location. CardTV-EPinv sizes, Hausdorff distances (HDs), and workflow duration were measured to assess interobserver variability and learning curve. Agreement between CardTV-EPinv was high using ADAS and Slicer3D workflows (HD 3.64 mm [2.7-4.5]). ADAS workflow was faster and more robust (ADAS 26 min [24-29] vs. Slicer3D 65 min [61-70], P < 0.001; system crashes: ADAS 0 vs. Slicer3D 7). In 20 patients (80% non-ischaemic cardiomyopathy, LVEF 35 ± 14%), 80 CardTV-EPinv were created using ADAS. CardTV-EPinv size was similar for both observers (11.8 mL [10.1-13.7] vs. 10.7 mL [9.6-11.8], P = 0.17), with high interobserver agreement (HD 1.68 mm [1.45-1.96]; 95th percentile HD < 4.8 mm [3.5-5.7]). Linear regression showed a steep learning curve for the student (P = 0.01). CONCLUSION: CardTV-EPinv creation showed excellent interobserver agreement and was faster and more robust using ADAS than 3D slicer. The steep learning curve appears clinically relevant given the limited use of STAR even in high-volume VT ablation centres.

• Keywords
• Ablation, Interobserver variability in imaging and EAM merging, STAR, Stereotactic arrhythmia radioablation, Ventricular tachycardia,
• MeSH
• Electrophysiologic Techniques, Cardiac MeSH
• Tachycardia, Ventricular * physiopathology   surgery   diagnostic imaging   radiotherapy   diagnosis   MeSH
• Learning Curve * MeSH
• Humans MeSH
• Observer Variation MeSH
• Radiotherapy Planning, Computer-Assisted * methods   MeSH
• Tomography, X-Ray Computed MeSH
• Predictive Value of Tests MeSH
• Workflow MeSH
• Radiosurgery * methods   MeSH
• Reproducibility of Results MeSH
• Software * MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

BACKGROUND: Idiopathic ventricular fibrillation (IVF) is a rare cause of sudden cardiac death and is a diagnosis of exclusion. With the availability of genetic testing, this condition is becoming increasingly rare. Nevertheless, in some cases, no identifiable cause is found. Management of recurrent IVF episodes poses a significant clinical challenge, often requiring advanced interventional approaches. CASE SUMMARY: We present a 43-year-old male with a history of out-of-hospital cardiac arrest due to VF in 2015. Despite extensive examinations, including normal coronary angiography, cardiac MRI, and genetic testing, no underlying aetiology was identified. The patient received an implantable cardioverter-defibrillator (ICD) for secondary prevention. After an 8-year arrhythmia-free period, he experienced recurrent ICD shocks in 2023. Repeated diagnostics, including MRI and genetic testing, yielded inconclusive results. An electrophysiological study revealed abnormalities in the Purkinje fibre network, including a focal source within the conduction system and a localized scar in the lower mid-left ventricular septum. Radiofrequency ablation targeting these areas successfully terminated the electrical storm. DISCUSSION: This case highlights the complexities in diagnosing and managing IVF, demonstrating a strong association between the Purkinje fibre network abnormalities in arrhythmogenesis. It underscores the importance of electrophysiological studies and catheter ablation in refractory cases, even when advanced imaging and genetic testing fail to reveal a clear aetiology. CONCLUSION: In patients with recurrent IVF refractory to conventional management, targeted ablation of Purkinje-related triggers not only terminates the storm, but provides durable rhythm control, as illustrated by our 8-month follow-up.

• Keywords
• Case report, Catheter ablation, Electrical storm, Idiopathic ventricular fibrillation, Purkinje network, Ventricular arrhythmia,
• Publication type
• Journal Article MeSH
• Case Reports MeSH

Up to one-third of patients referred for transcatheter tricuspid valve intervention (TTVI) have a transvalvular pacemaker (PPM) or implantable cardioverter-defibrillator (ICD) lead in place. Both the electrophysiology and interventional cardiology communities have been alerted to the complexity of decision-making in this situation due to potential interactions between the leads and the TTVI material, including the risk of jailing or damage to the leads. This document, commissioned by the European Heart Rhythm Association and the European Association of Percutaneous Cardiovascular Interventions of the ESC, reviews the scientific evidence to inform Heart Team discussions on the management of patients with a PPM or ICD who are scheduled for or have undergone TTVI.

• Keywords
• Transcatheter tricuspid valve intervention, cardiac implantable electronic device lead, lead extraction, lead jailing, tricuspid regurgitation,
• MeSH
• Heart Valve Prosthesis Implantation * adverse effects   standards   methods   instrumentation   MeSH
• Defibrillators, Implantable * standards   MeSH
• Pacemaker, Artificial * standards   MeSH
• Consensus MeSH
• Humans MeSH
• Risk Factors MeSH
• Cardiac Catheterization * adverse effects   standards   instrumentation   MeSH
• Tricuspid Valve * surgery   physiopathology   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Practice Guideline MeSH

Background and Objectives: Aortic stenosis (AS) is a frequent valvular disease characterized by the obstruction of left ventricular outflow. The resulting hemodynamic and structural changes create an arrhythmogenic substrate, with sudden cardiac death (SCD) often caused by ventricular arrhythmias (VAs) being a feared complication. This review examines the relationship between severe AS and VA, detailing the epidemiology, pathophysiological mechanisms, risk factors, and management approaches prior to aortic valve replacement (AVR). Materials and Methods: We conducted a comprehensive narrative review of the historical and contemporary literature investigating ventricular arrhythmias in severe aortic stenosis. Literature searches were performed in PubMed, MEDLINE, and Scopus databases using keywords, including "aortic stenosis", "ventricular arrhythmia", "sudden cardiac death", and "aortic valve replacement". Both landmark historical studies and modern investigations utilizing advanced monitoring techniques were included to provide a complete evolution of the understanding. Results: The prevalence of ventricular ectopy and non-sustained ventricular tachycardia increases with AS severity and symptom onset. Left ventricular hypertrophy, myocardial fibrosis, altered electrophysiological properties, and ischemia create the arrhythmogenic substrate. Risk factors include the male sex, concomitant aortic regurgitation, elevated filling pressures, and syncope. Diagnostic approaches range from standard electrocardiography to continuous monitoring and advanced imaging. Management centers on timely valve intervention, with medical therapy serving primarily as a bridge to AVR. Conclusions: Ventricular arrhythmias represent a consequence of valvular pathology in severe AS rather than an independent entity. Their presence signals advanced disease and a heightened risk for adverse outcomes. Multidisciplinary management with vigilant monitoring and prompt surgical referral is essential. Understanding this relationship enables clinicians to better identify high-risk patients requiring urgent intervention before life-threatening arrhythmic events occur.

• Keywords
• aortic stenosis, aortic valve disease, aortic valve replacement, sudden death, surgery, ventricular arrhythmia,
• MeSH
• Aortic Valve Stenosis * complications   surgery   physiopathology   MeSH
• Heart Valve Prosthesis Implantation * methods   MeSH
• Tachycardia, Ventricular etiology   MeSH
• Humans MeSH
• Death, Sudden, Cardiac etiology   MeSH
• Risk Factors MeSH
• Arrhythmias, Cardiac * etiology   physiopathology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH

Epicardial access during electrophysiology procedures offers valuable insights and therapeutic options for managing ventricular arrhythmias (VAs). The current clinical consensus statement on epicardial VA ablation aims to provide clinicians with a comprehensive understanding of this complex clinical scenario. It offers structured advice and a systematic approach to patient management. Specific sections are devoted to anatomical considerations, criteria for epicardial access and mapping evaluation, methods of epicardial access, management of complications, training, and institutional requirements for epicardial VA ablation. This consensus is a joint effort of collaborating cardiac electrophysiology societies, including the European Heart Rhythm Association, the Heart Rhythm Society, the Asia Pacific Heart Rhythm Society, the Latin American Heart Rhythm Society, and the Canadian Heart Rhythm Society.

• Keywords
• Cardiomyopathies, Catheter ablation, Clinical consensus statement, Electrophysiology procedures, Epicardial access, Ventricular arrhythmias, Ventricular fibrillation, Ventricular tachycardia,
• MeSH
• Electrophysiologic Techniques, Cardiac * standards   MeSH
• Epicardial Mapping * standards   MeSH
• Cardiology * standards   MeSH
• Catheter Ablation * standards   adverse effects   methods   MeSH
• Tachycardia, Ventricular * surgery   diagnosis   physiopathology   MeSH
• Consensus MeSH
• Humans MeSH
• Pericardium * surgery   physiopathology   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Practice Guideline MeSH
• Geographicals
• Europe MeSH

AIMS: Despite increasing prevalence, the general population lacks knowledge regarding diagnosis, implications, and management of cardiac arrhythmias (CA). This study aims to assess public perception of CA and identify knowledge gaps. METHODS AND RESULTS: The 36-item PULSE survey was disseminated via social media to the general population and conducted under the auspices of the European Heart Rhythm Association Scientific Initiatives Committee (EHRA SIC) with EHRA patient committee support. Among 3924 participants (2177 healthy, 1747 with previously diagnosed CA; 59% female, 90% European), 81% reported fear of CA. Females were more likely to be 'very' or 'moderately afraid' than males [odds ratio (OR) 1.159 (1.005, 1.337), P = 0.046]. While most recognized complications of CA-heart failure (82%), stroke (80%), and death (75%)-43% were unaware that CA can be asymptomatic. Those with cardiopulmonary resuscitation (CPR) training in the past 5 years were 2.6 times and 4.7 times more confident identifying sudden cardiac death and initiating CPR (P < 0.001). Confidence was lower in retired participants [OR 0.574 (0.499, 0.660), P < 0.001] and Southern Europeans [OR 0.703 (0.600, 0.824), P < 0.001]. Without CPR training, only 15% felt confident initiating CPR. Among CA participants, 28% reported severe to disabling daily symptoms. Males were more often asymptomatic (20% vs. 9%, P < 0.001). Treatment rates were comparable between sex categories (81% vs. 79%, P = 0.413). Interdisciplinary shared decision-making processes were reported by 4%. Notably, 1 in 10 CA cases was self-diagnosed using a wearable device, and 30% of CA participants used smartwatches for self-monitoring. CONCLUSION: Significant knowledge gaps regarding CA exist in the general population. Targeted educational initiatives could be a viable tool to enhance public knowledge, confidence in detecting and managing arrhythmias, particularly for women, who experience greater fear and symptom severity despite similar treatment rates.

• Keywords
• Atrial fibrillation, Awareness, Cardiac arrhythmias, Cardiac resuscitation, Pulse, Sudden cardiac death,
• MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Death, Sudden, Cardiac prevention & control   MeSH
• Surveys and Questionnaires MeSH
• Risk Factors MeSH
• Aged MeSH
• Sex Factors MeSH
• Social Media MeSH
• Arrhythmias, Cardiac * diagnosis   therapy   psychology   epidemiology   physiopathology   MeSH
• Fear MeSH
• Health Knowledge, Attitudes, Practice * MeSH
• Health Surveys MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Adolescent MeSH
• Young Adult MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Geographicals
• Europe epidemiology   MeSH

Stereotactic arrhythmia radioablation (STAR) is a novel, non-invasive, and promising treatment option for ventricular arrhythmias (VAs). It has been applied in highly selected patients mainly as bailout procedure, when (multiple) catheter ablations, together with anti-arrhythmic drugs, were unable to control the VAs. Despite the increasing clinical use, there is still limited knowledge of the acute and long-term response of normal and diseased myocardium to STAR. Acute toxicity appeared to be reasonably low, but potential late adverse effects may be underreported. Among published studies, the provided methodological information is often limited, and patient selection, target volume definition, methods for determination and transfer of target volume, and techniques for treatment planning and execution differ across studies, hampering the pooling of data and comparison across studies. In addition, STAR requires close and new collaboration between clinical electrophysiologists and radiation oncologists, which is facilitated by shared knowledge in each collaborator's area of expertise and a common language. This clinical consensus statement provides uniform definition of cardiac target volumes. It aims to provide advice in patient selection for STAR including aetiology-specific aspects and advice in optimal cardiac target volume identification based on available evidence. Safety concerns and the advice for acute and long-term monitoring including the importance of standardized reporting and follow-up are covered by this document. Areas of uncertainty are listed, which require high-quality, reliable pre-clinical and clinical evidence before the expansion of STAR beyond clinical scenarios in which proven therapies are ineffective or unavailable.

• Keywords
• Ablation, Radiotherapy, Stereotactic arrhythmia radioablation (STAR), Sudden death, Ventricular tachycardia,
• MeSH
• Action Potentials MeSH
• Cardiology * standards   MeSH
• Tachycardia, Ventricular * physiopathology   surgery   diagnosis   MeSH
• Consensus MeSH
• Humans MeSH
• Radiosurgery * adverse effects   standards   methods   MeSH
• Risk Factors MeSH
• Patient Selection * MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Practice Guideline MeSH
• Geographicals
• Europe MeSH

BACKGROUND: Charcot-Marie-Tooth is the most common inherited neuromuscular disorder. Rarely, it can be associated with heart failure and various arrhythmic disturbances. This case illustrates the challenges of making decisions to prevent sudden cardiac death in a patient with Charcot-Marie-Tooth disease. CASE SUMMARY: A 69-year-old male with a history of Type 1A Charcot-Marie-Tooth disease was admitted due to repetitive runs of ventricular tachycardia. Twelve-lead electrocardiogram, echocardiography, selective coronary angiography, and cardiac magnetic resonance did not clarify the cause of the electrical storm. As conservative therapy was not successful, radiofrequency ablation was chosen to treat the electrical storm. After this procedure, implantable cardioverter defibrillator (ICD) was implanted. The follow-up revealed severe perforation by the ventricular lead. An extraction was performed with no complications and a new lead was immediately implanted. The patient remains asymptomatic. Three episodes of non-sustained ventricular tachycardia were recorded during the last follow-up. DISCUSSION: This case illustrates the challenges of making decisions to prevent sudden cardiac death in a patient with Charcot-Marie-Tooth disease after successful ablation for electrical storm. Due to a lack of evidence, atypical origin of arrhythmia, and clinical presentation, we did not consider this as idiopathic arrhythmia and decided to implant an ICD, which was complicated by severe perforation by the lead. Specific recommendations for preventing sudden cardiac death in rare cardiac conditions, such as Charcot-Marie-Tooth disease, still need to be refined.

• Keywords
• Case report, Charcot–Marie–Tooth, Electric storm, ICD, Ventricular tachycardia,
• Publication type
• Journal Article MeSH
• Case Reports MeSH

AIMS: Recent-onset dilated cardiomyopathy (RODCM) is characterized by heterogeneous aetiology and diverse clinical outcomes, with scarce data on genotype-phenotype correlates. Our aim was to correlate individual RODCM genotypes with left ventricular reverse remodelling (LVRR) and clinical outcomes. METHODS AND RESULTS: In this prospective study, a total of 386 Czech RODCM patients with symptom duration ≤6 months underwent genetic counselling and whole-exome sequencing (WES). The presence of pathogenic (class 5) or likely pathogenic (class 4) variants in a set of 72 cardiomyopathy-related genes was correlated with the occurrence of all-cause death, heart transplantation, or implantation of a ventricular assist device (primary outcome) and/or ventricular arrhythmia event (secondary outcome). LVRR was defined as an improvement of left ventricular ejection fraction to >50% or ≥10% absolute increase, with a left ventricular end-diastolic diameter ≤33 mm/m2 or ≥10% relative decrease. Median follow-up was 41 months. RODCM was familial in 98 (25%) individuals. Class 4-5 variants of interest (VOIs) were identified in 125 (32%) cases, with 69 (18%) having a single titin-truncating variant (TTNtv) and 56 (14%) having non-titin (non-TTN) VOIs. The presence of class 4-5 non-TTN VOIs, but not of TTNtv, heralded a lower probability of 12-month LVRR and proved to be an independent baseline predictor both of the primary and the secondary outcome. The negative result of genetic testing was a strong protective baseline variable against occurrence of life-threatening ventricular arrhythmias. Detection of class 4-5 VOIs in genes coding nuclear envelope proteins was another independent predictor of both study outcomes at baseline and also of life-threatening ventricular arrhythmias after 12 months. Class 4-5 VOIs of genes coding cytoskeleton were associated with an increased risk of life-threatening ventricular arrhythmias after baseline assessment. A positive family history of dilated cardiomyopathy alone only related to a lower probability of LVRR at 12 months and at the final follow-up. CONCLUSIONS: RODCM patients harbouring class 4-5 non-TTN VOIs are at higher risk of progressive heart failure and life-threatening ventricular arrhythmias. Genotyping may improve their early risk stratification at baseline assessment.

• Keywords
• Genetics, Left ventricular reverse remodelling, Prognosis, Recent‐onset dilated cardiomyopathy, Whole‐exome sequencing,
• MeSH
• Cardiomyopathy, Dilated * genetics   physiopathology   MeSH
• Adult MeSH
• Ventricular Function, Left physiology   MeSH
• Genotype * MeSH
• Middle Aged MeSH
• Humans MeSH
• Follow-Up Studies MeSH
• Prospective Studies MeSH
• Ventricular Remodeling * genetics   physiology   MeSH
• Exome Sequencing MeSH
• Stroke Volume physiology   MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Geographicals
• Czech Republic epidemiology   MeSH

BACKGROUND AND AIMS: Risk stratification of sudden cardiac death after myocardial infarction and prevention by defibrillator rely on left ventricular ejection fraction (LVEF). Improved risk stratification across the whole LVEF range is required for decision-making on defibrillator implantation. METHODS: The analysis pooled 20 data sets with 140 204 post-myocardial infarction patients containing information on demographics, medical history, clinical characteristics, biomarkers, electrocardiography, echocardiography, and cardiac magnetic resonance imaging. Separate analyses were performed in patients (i) carrying a primary prevention cardioverter-defibrillator with LVEF ≤ 35% [implantable cardioverter-defibrillator (ICD) patients], (ii) without cardioverter-defibrillator with LVEF ≤ 35% (non-ICD patients ≤ 35%), and (iii) without cardioverter-defibrillator with LVEF > 35% (non-ICD patients >35%). Primary outcome was sudden cardiac death or, in defibrillator carriers, appropriate defibrillator therapy. Using a competing risk framework and systematic internal-external cross-validation, a model using LVEF only, a multivariable flexible parametric survival model, and a multivariable random forest survival model were developed and externally validated. Predictive performance was assessed by random effect meta-analysis. RESULTS: There were 1326 primary outcomes in 7543 ICD patients, 1193 in 25 058 non-ICD patients ≤35%, and 1567 in 107 603 non-ICD patients >35% during mean follow-up of 30.0, 46.5, and 57.6 months, respectively. In these three subgroups, LVEF poorly predicted sudden cardiac death (c-statistics between 0.50 and 0.56). Considering additional parameters did not improve calibration and discrimination, and model generalizability was poor. CONCLUSIONS: More accurate risk stratification for sudden cardiac death and identification of low-risk individuals with severely reduced LVEF or of high-risk individuals with preserved LVEF was not feasible, neither using LVEF nor using other predictors.

• Keywords
• Implantable cardioverter-defibrillator, Myocardial infarction, Primary prevention, Sudden cardiac death,
• MeSH
• Defibrillators, Implantable * MeSH
• Electrocardiography MeSH
• Risk Assessment methods   MeSH
• Myocardial Infarction * mortality   complications   MeSH
• Humans MeSH
• Death, Sudden, Cardiac * prevention & control   epidemiology   etiology   MeSH
• Stroke Volume * physiology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Meta-Analysis MeSH