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Background Brugada syndrome (BrS) has been associated with sudden cardiac death in otherwise healthy subjects, and drug‐induced BrS accounts for 55% to 70% of all patients with BrS. This study aims to develop a deep convolutional neural network and evaluate its performance in recognizing and predicting BrS diagnosis. Methods and Results Consecutive patients who underwent ajmaline testing for BrS following a standardized protocol were included. ECG tracings from baseline and during ajmaline were transformed using wavelet analysis and a deep convolutional neural network was separately trained to (1) recognize and (2) predict BrS type I pattern. The resultant networks are referred to as BrS‐Net. A total of 1188 patients were included, of which 361 (30.3%) patients developed BrS type I pattern during ajmaline infusion. When trained and evaluated on ECG tracings during ajmaline, BrS‐Net recognized a BrS type I pattern with an AUC‐ROC of 0.945 (0.921–0.969) and an AUC‐PR of 0.892 (0.815–0.939). When trained and evaluated on ECG tracings at baseline, BrS‐Net predicted a BrS type I pattern during ajmaline with an AUC‐ROC of 0.805 (0.845–0.736) and an AUC‐PR of 0.605 (0.460–0.664). Conclusions BrS‐Net, a deep convolutional neural network, can identify BrS type I pattern with high performance. BrS‐Net can predict from baseline ECG the development of a BrS type I pattern after ajmaline with good performance in an unselected population.

Introduction: The ablation of ventricular tachycardia, including premature ventricular contractions, is an approved, albeit infrequent procedure in pediatric patients. Data are scarce regarding the outcomes of this procedure. The purpose of this study was to share a high-volume center experience and patient outcomes for catheter ablation of ventricular ectopy and ventricular tachycardia in pediatric population. Methods: Data were retrieved from the institutional data bank. Outcomes over time were evaluated, and procedural details were compared. Results: A total of 116 procedures were performed on 102 pediatric patients between July 2009 and May 2021 at the Rajaie Cardiovascular Medical and Research Center in Tehran, Iran. Ablation was not performed in 4 procedures (3.4%) due to high-risk substrates. Of the remaining 112 ablations performed, 99 (88.4%) were successful. However, one patient died due to a coronary complication. There were no significant differences observed in early ablation results based on patients' age, sex, cardiac anatomy, or ablation substrates (P > 0.05). Follow-up records were available for 80 procedures, and 13 (16.3%) of those experienced recurrence. During long-term follow-up, none of the variables mentioned above were statistically different between patients with or without arrhythmia recurrence. Conclusion: The overall success rate of pediatric ventricular arrhythmia ablation is favorable. We found no significant predictor for the procedural success rate concerning acute and late outcomes. Larger multicenter studies are needed to elucidate the predictors and outcomes of the procedure.

Brugada syndrome (BrS) is an inherited cardiac channelopathy with variable expressivity that can lead to sudden cardiac arrest (SCA). Studies worldwide suggest that BrS and Brugada pattern (BrP) have low prevalences in general. However, studies also note that BrS is most prevalent among certain Asian populations. Among the different global regions, the highest prevalence is believed to be in Southeast Asia, followed by the Middle East, South Asia, East Asia, Europe, and North America. It is not only important to recognize such varying degrees of BrS prevalence within Asia but also to understand that there may be significant differences in terms of presenting symptoms, occult risk factors, and the impact on clinical outcomes. The importance of identifying such differences lies in the necessity to develop improved risk assessment strategies to guide secondary prevention and treatment for these patients. Specifically, the decision to pursue placement of an implantable cardiac defibrillator (ICD) can be lifesaving for high-risk BrS patients. However, there remains a significant lack of consensus on how to best risk stratify BrS patients. While the current guidelines recommend ICD implantation in patients with spontaneous Type 1 ECG pattern BrS who present with syncope, there may still exist additional clinical factors that may serve as better predictors or facilitate more refined risk stratification before malignant arrhythmias occur. This carries huge relevance given that BrS patients often do not have any preceding symptoms prior to SCA. This review seeks to delineate the differences in BrS presentation and prevalence within the Asian continent in the hope of identifying potential risk factors to guide better prognostication and management of BrS patients in the future.

Resumo Trinta anos atrás, uma nova síndrome clínico-eletrocardiográfica distinta foi descrita, agora conhecida como síndrome de Brugada (SBr). Típico para essa síndrome é o eletrocardiograma com supradesnivelamento do segmento ST nas derivações precordiais direitas. A apresentação clínica da doença é altamente variável: os pacientes podem permanecer completamente assintomáticos, mas também podem desenvolver episódios de síncope, fibrilação atrial (FA), síndrome do nódulo sinusal (SNS), distúrbios de condução, assistolia e fibrilação ventricular (FV). A doença é causada por mutações nos genes responsáveis pelo potencial de ação das células do coração. O gene mais frequentemente envolvido é o SCN5A, que controla a estrutura e função do canal de sódio cardíaco. A descrição dessa nova síndrome teve implicações muito positivas em todos os campos da medicina.

Abstract Aims To evaluate the clinical outcome in patients undergoing repeat procedures for recurrent persistent atrial fibrillation following an index cryoballoon (CB‐A) pulmonary vein isolation ablation on a mid‐term follow‐up of 12 months. Methods In this propensity score‐matched comparison, 50 patients undergoing left atrial posterior wall isolation (LAPWI) with the CB‐A were matched to 50 patients treated with additional linear ablation using radiofrequency catheter ablation (RFCA). Results Meantime to repeat the procedure was 9.74 ± 4.36 months. At 12 months follow‐up freedom from atrial tachyarrhythmias (ATas) was achieved in 82% of patients in the LAPWI group and in 62% of patients in the linear ablation group (P = .03). Regression analysis demonstrated that relapses during the blanking period and LA dimensions were independent predictors of ATas recurrences following the repeat procedure. Conclusion LAPWI using CB‐A is associated with a significantly higher freedom from atrial arrhythmias when compared with the RFCA mediated left atrial linear lesions on a mid‐term follow‐up of 12 months in patients with persAF undergoing a redo procedure.

Brugada syndrome is a rare hereditary arrhythmia disorder characterized by a distinctive electrocardiogram pattern and an elevated risk of ventricular arrhythmias and sudden cardiac death in young adults. Despite recent advances, it remains a complex condition, encompassing mechanisms, genetics, diagnosis, arrhythmia risk stratification, and management. The underlying electrophysiological mechanism of Brugada syndrome requires further investigation, with current theories focusing on abnormalities in repolarization, depolarization, and current-load match. The genetic basis of the syndrome is strong, with mutations found in genes encoding subunits of cardiac sodium, potassium, and calcium channels, as well as genes involved in channel trafficking and regulation. While the initial discovery of mutations in the SCN5A gene provided valuable insights, Brugada syndrome is now recognized as a multifactorial disease influenced by several loci and environmental factors, challenging the traditional autosomal dominant inheritance model. This comprehensive review aims to provide a current understanding of Brugada syndrome, focusing on its pathophysiology, genetic mechanisms, and novel models of risk stratification. Advancements in these areas hold the potential to facilitate earlier diagnosis, improve risk assessments, and enable more targeted therapeutic interventions.

Background Pharmacological treatment of atrial fibrillation (AF) in the setting of Brugada syndrome (BrS) is challenging. In addition, patients with BrS with an implantable cardioverter‐defibrillator (ICD) might experience inappropriate shocks for fast AF. Long‐term outcome of pulmonary vein isolation in BrS has not been well established yet, and it is still unclear whether pulmonary vein triggers are the only pathophysiological mechanism of AF in BrS. The aim of the study is to assess the long‐term outcomes in patients with BrS undergoing pulmonary vein isolation for paroxysmal AF compared with a matched cohort of patients without BrS. Methods and Results Sixty patients with BrS undergoing pulmonary vein isolation with cryoballoon catheter ablation for paroxysmal AF were matched with 60 patients without BrS, who underwent the same procedure. After a mean follow‐up of 58.2±31.7 months, freedom from atrial tachyarrhythmias was achieved in 61.7% in the BrS group and in 78.3% in the non‐BrS group (log‐rank P=0.047). In particular, freedom from AF was 76.7% in the first group and in 83.3% in the second (P=0.27), while freedom from atrial tachycardia/atrial flutter was 85% and 95% (P=0.057). In the BrS group, 29 patients (48.3%) had an ICD and 8 (27.6%) had a previous ICD‐inappropriate shock for fast AF. In the BrS cohort, ICD‐inappropriate interventions for AF were significantly reduced after ablation (3.4% versus 27.6%; P=0.01). Conclusions Pulmonary vein isolation in patients with BrS was associated with higher rate of arrhythmic recurrence. Despite this, catheter ablation significantly reduced inappropriate ICD interventions in BrS patients and can be considered a therapeutic strategy to prevent inappropriate device therapies.

BackgroundNew technologies for ablation procedures are often produced by different companies with no cross-compatibility out of the box. This is not a negligible clinical problem since those separately developed devices are often used together. The aim of this study was to develop a bench-testing method to assess compatibility between the DiamondTemp ablation system (DTA) and the Rhythmia electroanatomic mapping system (EAM).MethodsDifferent setups were tested. DTA was connected to the Rhythmia EAM using the following configurations: 3.1. An Ensite EPT GenConnect box (GCB) and Rhythmia Maestro GCB (Maestro GCB, native Rhythmia setup); 3.2. The Medtronic GCB-E and Maestro GCB; 3.3. The Medtronic GCB-E out via the Medtronic GCB-E directly to the Rhythmia at box 1 (pin A61 to A64).ResultsThe DTA location was represented in real-time on the Rhythmia EAM. A proper tracking of the DTA was observed in all setups tested by visual comparison of physical catheter movements and its representation on EAM. In configuration 3.1, a significant shift was observed after the first radio frequency (RF) application; however, further applications caused no further shift. In setup 3.2, no significant shift was observed. The setup 3.3 showed a massive shift in the catheter position before ablation compared to baseline points acquired using the Orion catheter as a reference.ConclusionsA universal and reproducible solution for compatibility testing between the various mapping systems and the ablation catheters has been described. DTA has been demonstrated as compatible with Rhythmia EAM with satisfactory results if a specific setup is used.

BackgroundCoronavirus disease 2019 (COVID-19) is a systemic disease caused by severe acute respiratory syndrome coronavirus 2. Arrhythmias are frequently associated with COVID-19 and could be the result of inflammation or hypoxia. This study aimed to define the incidence of arrhythmias in patients with COVID-19 and to correlate arrhythmias with pulmonary damage assessed by computed tomography (CT).MethodsAll consecutive patients with a COVID-19 diagnosis hospitalized at Universitair Ziekenhuis Brussel, Belgium, between March 2020 and May 2020, were screened. All included patients underwent a thorax CT scan and a CT severity score, a semiquantitative scoring system of pulmonary damage, was calculated. The primary endpoint was the arrhythmia occurrence during follow-up.ResultsIn this study, 100 patients were prospectively included. At a mean follow-up of 19.6 months, 25 patients with COVID-19 (25%) experienced 26 arrhythmic episodes, including atrial fibrillation in 17 patients, inappropriate sinus tachycardia in 7 patients, atrial flutter in 1 patient, and third-degree atrioventricular block in 1 patient. No ventricular arrhythmias were documented. Patients with COVID-19 with arrhythmias showed more often need for oxygen, higher oxygen maximum flow, longer QTc at admission, and worse damage at CT severity score. In univariate logistic regression analysis, significant predictors of the primary endpoint were: the need for oxygen therapy (odds ratio [OR] 4.59, 95% CI 1.44–14.67, p = 0.01) and CT severity score of pulmonary damage (OR per 1 point increase 1.25, 95% CI 1.11–1.4, p < 0.001).ConclusionsIn a consecutive cohort of patients with COVID-19 the incidence of cardiac arrhythmias was 25%. The need for oxygen therapy and CT severity score were predictors of arrhythmia occurrence during follow-up.

Abstract Background Brugada syndrome (BrS) is somewhat a challenging diagnosis, due to its dynamic pattern. One of the aspects of this disease is a significant conduction disorder located in the right ventricular outflow tract (RVOT), which can be explained as a consequence of low expression of Connexin‐43. This decreased conduction speed is responsible for the typical electrocardiographic pattern. Opposite leads located preferably in inferior leads of the electrocardiogram may show a deep and widened S wave associated with ascending ST segment depression. Holter monitoring electrocardiographic (ECG) aspects is still a new frontier of knowledge in BrS, especially in intermittent clinical presentations. Methods We describe, as an exploratory analysis, five case series of intermittent type 1 BrS to demonstrate the appearance of ascending ST segment depression and widening of the S wave, during 3‐channel 24h‐Holter monitoring (C1, C2 and C3) with bipolar leads. Results In the five cases described, the ST segment depression was observed mainly in C2, but in some cases also in C1 and C3. Only case 1 presented concomitant intermittent elevation of the ST segment in C1. All cases were intermittent. Conclusion The recognition of an ECG pattern with ascending ST‐segment depression and widening of the S wave in 3‐channel Holter described in this case series should raise a suspicion of the BrS and suggests the counterpart of a dromotropic disturbance registered in the RVOT and/or reciprocal changes.

Conduction delay in the right ventricular outflow tract as manifested in the electrocardiogram constitutes a high-risk predictor of ventricular arrhythmias in patients with Brugada syndrome. We present a case with a right QRS axis between –90° and ±180°. This feature has never been reported in the context of Brugada syndrome. (Level of Difficulty: Advanced.)

Background: Clinical management of vaso-vagal syncope (VVS) remains challenging since no therapy has proven to completely prevent VVS recurrence. Objective: The purpose of this study was to analyze the mid-term outcome of cryoballoon (CB) cardioneuroablation achieved by pulmonary vein isolation (PVI) in patients with VVS. Methods: Patients who underwent CB cardioneuroablation in our centers between January 2014 to June 2018 were included. All patients had a history of VVS or pre-syncope despite therapeutic attempts with medical and/or pacing treatments. Patients were excluded in case of structural heart diseases, cerebrovascular diseases or suspected drug-related syncope. Both heart rate (HR) and atrio-ventricular (AV) interval were analyzed on the 12-lead electrocardiogram (ECG) the day before the procedure, the day after, and in the follow-up. Results: In total, 26 patients (76.9% males, 37.5 ± 9.0 years old) were included. All patients underwent a successful procedure with the 28 mm second-generation Arctic Front Advance CB. No major complication occurred. At a mean follow-up of 20.1 ± 11.6 months the freedom from VVS or reflex pre-syncope was 83,7%, with 22 patients free from any clinical recurrence. Basal HR significantly increased the day after the procedure (57.2 bpm vs 78.3 bpm, p

Abstract Background The prevalence and the clinical impact of conversion of atrial fibrillation (AF) to sinus rhythm (SR) during cryoballoon ablation (CB‐A) are unknown. Objective The purpose of this study was to evaluate the prevalence of restoration of SR during CB‐A and the clinical impact of this phenomenon. Methods Between January 2012 and September 2018, all patients who experienced conversion of AF to SR during CB‐A were included. This group was subsequently matched for gender, age, type of AF, diagnosis‐to‐ablation time, and left atrial size with patients who underwent CB‐A and did not experienced conversion of AF to SR. After discharge, patients were scheduled for follow‐up visits at 1, 3, 6, and 12 months and 24 hours Holter recordings were obtained at each follow‐up visit. All documented AF episodes of >30 seconds were considered as recurrence. A 3 month post‐procedural blanking period (BP) was applied. Results A total of 1559 patients underwent pulmonary veins isolation by CB‐A between January 2012 and September 2018; among them, 58 patients (3.7%) experienced restoration of SR during CB‐A. In total, 53 patients (41 males [77.3%], mean age 61.4 ± 13.3 years) were included in the case group. During CB‐A, restoration of SR occurred more frequently during right‐side PVs applications (right inferior pulmonary vein 39.6%, right superior pulmonary vein 30.2%). If considering a BP, at 2 year follow‐up, freedom from recurrences was 86.5% in the case group and 68.0% in the control group (P = .036). Conclusion Conversion of AF to SR is a favorable and relatively frequent phenomenon during cryoballoon pulmonary vein isolation ablation.

Background The rate of sudden cardiac death (SCD) in Brugada syndrome (BrS) is ≈1%/y. Noninvasive electrocardiographic imaging is a noninvasive mapping system that has a role in assessing BrS depolarization and repolarization abnormalities. This study aimed to analyze electrocardiographic imaging parameters during ajmaline test (AJT). Methods and Results All consecutive epicardial maps of the right ventricle outflow tract (RVOT‐EPI) in BrS with CardioInsight were retrospectively analyzed. (1) RVOT‐EPI activation time (RVOT‐AT); (2) RVOT‐EPI recovery time, and (3) RVOT‐EPI activation‐recovery interval (RVOT‐ARI) were calculated. ∆RVOT‐AT, ∆RVOT‐EPI recovery time, and ∆RVOT‐ARI were defined as the difference in parameters before and after AJT. SCD‐BrS patients were defined as individuals presenting a history of aborted SCD. Thirty‐nine patients with BrS were retrospectively analyzed and 12 patients (30.8%) were SCD‐BrS. After AJT, an increase in both RVOT‐AT [105.9 milliseconds versus 65.8 milliseconds, P

Electrical isolation of pulmonary veins (PVI) is the cornerstone of invasive treatment of atrial fibrillation (AF). However, arrhythmia-free survival of a PVI only approach is suboptimal in patients with persistent and long-term persistent AF. Hybrid AF ablation has been developed with the aim of combining the advantages of a thoracoscopic surgical ablation (direct visualization of anatomical structures to be spared and the possibility to perform epicardial lesions) and endocardial ablation (possibility to check line block, confirm PVI, and possibility to perform cavotricuspid isthmus ablation). Patient selection is of utmost importance. In persistent and long-term persistent AF, hybrid AF ablation demonstrated promising results in terms of AF free survival. It has been associated with a relatively low complication rate if performed in centers with expertise in hybrid procedures and experience with both surgical and endocardial ablation. Different techniques have been described, with different approaches and lesion sets. The aim of this review is to provide a state-of-the-art overview of hybrid AF ablation.

Preventing sudden cardiac death (SCD) in athletes is a primary duty of sports cardiologists. Current recommendations for detecting high-risk cardiovascular conditions (hr-CVCs) are history and physical examination (H&P)-based. We discuss the effectiveness of H&P-based screening versus more-modern and accurate methods. In this position paper, we review current authoritative statements and suggest a novel alternative: screening MRI (s-MRI), supported by evidence from a preliminary population-based study (completed in 2018), and a prospective, controlled study in military recruits (in development).We present: 1. Literature-Based Comparisons (for diagnosing hr-CVCs): Two recent studies using traditional methods to identify hr-CVCs in >3,000 young athletes are compared with our s-MRI-based study of 5,169 adolescents. 2. Critical Review of Previous Results: The reported incidence of SCD in athletes is presently based on retrospective, observational, and incomplete studies. H&P’s screening value seems minimal for structural heart disease, versus echocardiography (which improves diagnosis for high-risk cardiomyopathies) and s-MRI (which also identifies high-risk coronary artery anomalies). Electrocardiography is valuable in screening for potentially high-risk electrophysiological anomalies. 3. Proposed Project: We propose a prospective, controlled study (2 comparable large cohorts: one historical, one prospective) to compare: (1) diagnostic accuracy and resulting mortality-prevention performance of traditional screening methods versus questionnaire/electrocardiography/s-MRI, during 2-month periods of intense, structured exercise (in military recruits, in advanced state of preparation); (2) global costs and cost/efficiency between these two methods. This study should contribute significantly toward a comprehensive understanding of the incidence and causes of exercise-related mortality (including establishing a definition of hr-CVCs) while aiming to reduce mortality.

Background: The new version of inner lumen mapping catheter (Achieve Advance™; Medtronic, Minnesota, USA) includes a new solid core which provides improved rotational response, as compared to the current Achieve Mapping Catheter. In the present study, we sought to analyze the rate of visualisation of real-time recordings using this new device comparing it with a large cohort of patients having undergone second generation cryoballoon (CB) ablation using the previous Achieve mapping catheter. Methods: All patients having undergone CB ablation using the Achieve Advance and the last 150 consecutive patients having undergone CB ablation using the previous Achieve were analysed. Exclusion criteria were presence of an intracavitary thrombus, uncontrolled heart failure, moderate or severe valvular disease and contraindications to general anesthesia. Results: A total of 200 consecutive patients (60.1 ± 9.5 years, 75% males) were evaluated (50 Achieve Advance and 150 old Achieve). Real-time recordings were significantly more prevalent in the “new Achieve Advance” population compared with the “old Achieve” group (73.5% vs 56.8%; p = 0.0001). Real-time recordings could be more frequently visualized in the “Achieve Advance” group in all veins except RIPV (LSPV: 86% vs 71.3%, p = 0.04; LIPV: 84% vs 62.7%, p = 0.005; RSPV: 78% vs 52%, p

Abstract Background Radiofrequency (RF) ablation is a well‐established approach to treat premature ventricular contractions (PVC) and is associated with good outcomes. Aim The present study sought to analyze the acute efficacy and 1‐year outcomes of PVC ablation using RF technology with an approach based on automated pace‐mapping and contact force (CF) information. Methods Sixty‐one consecutive patients (52.4% males, age 45.9 ± 12.5) underwent catheter ablation for symptomatic monomorphic PVC. All procedures were guided by a 3‐dimensional mapping system; site of ablation was selected based on PASO™ aided pace‐mapping; RF was started on the selected location when stable catheter position with >10 g of CF were obtained. The procedure was defined as acutely effective if the PVC was eliminated and it did not recur during within 30 minutes. Long‐term efficacy was defined as a decrease by more than 95% at 1 year of the initial PVC burden at ECG Holter monitoring. Results The PVC ablation was performed in the right ventricular outflow tract in 37 patients (60.7%), left ventricle in 15 patients (24.6%), coronary cusps in 6 patients (9.8%), right ventricle in 3 patients (4.9%); PVC ablation was acutely successful in 59 of patients (96.7%). At 1‐year efficacy was obtained in 57 patients (93.4%). No major complications occurred. Mean procedural and fluoroscopy time were 94.5 ± 20.9 and 4.3 ± 2.5 minutes respectively. Conclusion Premature ventricular contraction RF ablation mainly guided by PASO™ and CF showed high success rate in both acute and 1‐year follow‐up (96.7% and 93.4% respectively). The best efficacy cut‐off for RF ablation of PVCs has been identified in presence of both PASO™ ≥95% and CF >10 g.

Abstract Background Brugada syndrome is an ion channelopathy that predisposes affected subjects to ventricular tachycardia/fibrillation (VT/VF), potentially leading to sudden cardiac death (SCD). Tpeak‐Tend intervals, (Tpeak‐Tend)/QT ratio and Tpeak‐Tend dispersion have been proposed for risk stratification, but their predictive values in Brugada syndrome have been challenged recently. Methods A systematic review and meta‐analysis was conducted to examine their values in predicting arrhythmic and mortality outcomes in Brugada Syndrome. PubMed and Embase databases were searched until 1 May 2018, identifying 29 and 57 studies. Results Nine studies involving 1740 subjects (mean age 45 years old, 80% male, mean follow‐up duration was 68 ± 27 months) were included. The mean Tpeak‐Tend interval was 98.9 ms (95% CI: 90.5‐107.2 ms) for patients with adverse events (ventricular arrhythmias or SCD) compared to 87.7 ms (95% CI: 80.5‐94.9 ms) for those without such events, with a mean difference of 11.9 ms (95% CI: 3.6‐20.2 ms, P = 0.005; I2 = 86%). Higher (Tpeak‐Tend)/QT ratios (mean difference = 0.019, 95% CI: 0.003‐0.036, P = 0.024; I2 = 74%) and Tpeak‐Tend dispersion (mean difference = 7.8 ms, 95% CI: 2.1‐13.4 ms, P = 0.007; I2 = 80%) were observed for the event‐positive group. Conclusion Tpeak‐Tend interval, (Tpeak‐Tend)/QT ratio and Tpeak‐Tend dispersion were higher in high‐risk than low‐risk Brugada subjects, and thus offer incremental value for risk stratification.

Background Short ECG P‐wave duration has recently been demonstrated to be associated with higher risk of atrial fibrillation (AF). The aim of this study was to assess the rate of AF recurrence after pulmonary vein isolation in patients with a short P wave, and to mechanistically elucidate the observation by computer modeling. Methods and Results A total of 282 consecutive patients undergoing a first single‐pulmonary vein isolation procedure for paroxysmal or persistent AF were included. Computational models studied the effect of adenosine and sodium conductance on action potential duration and P‐wave duration (PWD). About 16% of the patients had a PWD of 110 ms or shorter (median PWD 126 ms, interquartile range, 115 ms–138 ms; range, 71 ms–180 ms). At Cox regression, PWD was significantly associated with AF recurrence (P=0.012). Patients with a PWD

Vasovagal syncope is characterized by vasodilatation and/or bradycardia and thereby a fall in arterial BP and global cerebral perfusion in response to a trigger. Although it is a benign condition, patients with frequent and traumatic episodes need treatment in order to improve quality of life. We describe the case of a 17-years-old boy suffering from cardioinhibitory syncope. At the end of a complete negative cardiac and neurological examination, a loop recorder was implanted. During the subsequent follow-up the ILR documented a 9-s pause. To improve the patient's compliance, and considering cardioinhibitory syncope as a temporary condition, a leadless pacemaker was eventually implanted.

Brugada syndrome is characterized by typical electrocardiogram changes and a high risk for sudden cardiac death (Priori et al., 2013). In addition to the well known electrical substrate, morphological and functional alterations appeared to be present in a subset of the Brugada syndrome patients (Catalano et al., 2009). Echocardiographic speckle tracking enables us to detect subtle contraction alterations (Smiseth et al.,2016). We performed transthoracic echocardiography with speckle tracking analysis in 82 healthy controls and 175 Brugada syndrome patients. Main findings are presented and discussed in the article “Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias” (Scheirlynck et al., 2019). This related Data article contains segmental longitudinal strain values for RV and LV, and the comparison of echocardiographic parameters between Brugada syndrome patients with spontaneous and drug-induced type 1 pattern and between patients with and without ventricular arrhythmia inducibility during electrophysiological study. Keywords: Brugada syndrome, Inducible, Mechanical dispersion, Strain, Speckle tracking echocardiography, Spontaneous type 1

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a primary electrical disease characterized by a normal resting electrocardiogram and induction of malignant arrhythmias during adrenergic stress leading to syncope or sudden cardiac death (SCD). CPVT is caused by mutations in the cardiac ryanodine receptor (RyR2) or in the sarcoplasmic reticulum protein calsequestrin 2 genes (CASQ2). The RyR2 mutations are responsible for the autosomal dominant form of CPVT, while CASQ2 mutations are rare and account for the recessive form. These mutations cause a substantial inballance in the homeostasis of intracellular calcium resulting in polymorphic ventricular tachycardia through triggered activity. Beta blockers were for years the cornerstone of therapy in these patients. Sodium channel blockers, especially flecainide, have an additive role in those not responding in beta blockade. Implantation of defibrillators needs a meticulous evaluation since inappropriate shocks may lead to electrical storm. Finally, cardiac sympathetic denervation might also be an alternative therapeutic option. Early identification and risk stratification is of major importance in patients with CPVT. The aim of the present review is to present the arrhythmogenic mechanisms of the disease, the current therapies applied and potential future perspectives.

Aim: Peri-procedural thromboembolic (TE) and hemorrhagic events are complications of major concern for patients undergoing cryoballoon (CB) ablation for atrial fibrillation (AF). While peri-procedural anticoagulation management could decrease the incidence of these complications, data on CB ablation are scarce. The role of novel oral anticoagulants (NOACs) has not been thoroughly tested in this population. Methods: In the present study, we sought to assess acute peri-procedural complications in patients undergoing CB ablation for AF under different anticoagulation regimens; anticoagulation administration was performed according to the CHA2DS2-VASc score guidelines. To the best of our knowledge, this is the first study that compares 1) uninterrupted warfarin, 2) bridging therapy with low molecular weight heparin (LMWH), 3) aspirin and 4) NOACs in this subgroup of patients. Results: NOACs were as effective as uninterrupted warfarin in terms of bleeding complications and TE events. Surprisingly, the aspirin group had more hemorrhagic complications than both the warfarin and NOACs groups. Conclusion: In the current study, the use of NOACs was an effective and safe approach in CB ablation.

During last centuries, Early Repolarization pattern has been interpreted as an ECG manifestation not linked to serious cardiovascular events. This view has been challenged on the basis of sporadic clinical observations that linked the J-wave with ventricular arrhythmias and sudden cardiac death. The particular role of this characteristic pattern in initiating ventricular fibrillation has been sustained by clinical descriptions of a marked and consistent J-wave elevation preceding the onset of the ventricular arrhythmia. Until now, Early Repolarization syndrome patients have been evaluated using ECG and theorizing different interpretations of the findings. Nonetheless, ECG analysis is not able to reveal all depolarization and repolarization properties and the explanation for this clinical events. Recent studies have characterized the epicardial substrate in these patients on the basis of high-resolution data, in an effort to provide insights into the substrate properties that support arrhythmogenicity in these patients. An overview for the current evidence supporting different theories explaining Early Repolarization Syndrome is provided in this review. Finally, future developments in the field directed toward individualized treatment strategies are examined.

We present the case of a 50-year-old patient with several episodes of syncope and documented simultaneous wide and narrow QRS complex tachycardia. We then review this tacharrhythmia, focusing on electrophysiological findings and pathophysiology, diagnosis and treatment. Resumo: Apresentamos um caso de um doente de 50 anos com diversos episódios de síncope e documentação de taquicardia com complexo QRS simultaneamente largo e estreito. Fazemos uma revisão desta taquiarritmia tendo em especial atenção achados electrofisiológicos e fisiopatologia, diagnóstico e tratamento. Keywords: Atrioventricular nodal reentrant tachycardia, Right ventricular outflow tract tachycardia, Double tachycardia, Spontaneous entrainment, Palavras-chave: Taquicardia de reentrada nodal auriculoventricular, Taquicardia do tracto de saída do ventrículo direito, Taquicardia dupla, Arrastamento espontâneo

Catheter ablation is an accepted, highly effective modality of treatment for cardiac arrhythmias in children. The success rate depends on the operator’s experience, especially in cases involving complex anatomies. We hereby report our recent experience of successful ablation of accessory pathways in three children with complex congenital heart diseases. The first case was a 7-year-old girl with tricuspid atresia and a previous Glenn shunt, in whom a sub-epicardial overt accessory pathway was successfully ablated via the coronary sinus. The second case, a 9-year-old girl, received accessory pathway ablation via the fenestration of an extracardiac Fontan pathway. The third case was a 14-year-old boy with dextrocardia, common atrium, common ventricle, and a previous extracardiac Fontan operation, in whom ablation of a concealed accessory pathway was carried out retrogradely from the aorta. All the ablations were done in Rajaie Cardiovascular, Medical and Research Center, Tehran, and all the patients were discharged from the hospital without any complication.

The use of next-generation sequencing enables a rapid analysis of many genes associated with sudden cardiac death in diseases like Brugada Syndrome. Genetic variation is identified and associated with 30-35% of cases of Brugada Syndrome, with nearly 20-25% attributable to variants in SCN5A, meaning many cases remain undiagnosed genetically. To evaluate the role of genetic variants in arrhythmogenic diseases and the utility of next-generation sequencing, we applied this technology to resequence 28 main genes associated with arrhythmogenic disorders.A cohort of 45 clinically diagnosed Brugada Syndrome patients classified as SCN5A-negative was analyzed using next generation sequencing. Twenty-eight genes were resequenced: AKAP9, ANK2, CACNA1C, CACNB2, CASQ2, CAV3, DSC2, DSG2, DSP, GPD1L, HCN4, JUP, KCNE1, KCNE2, KCNE3, KCNH2, KCNJ2, KCNJ5, KCNQ1, NOS1AP, PKP2, RYR2, SCN1B, SCN3B, SCN4B, SCN5A, SNTA1, and TMEM43. A total of 85 clinically evaluated relatives were also genetically analyzed to ascertain familial segregation.Twenty-two patients carried 30 rare genetic variants in 12 genes, only 4 of which were previously associated with Brugada Syndrome. Neither insertion/deletion nor copy number variation were detected. We identified genetic variants in novel candidate genes potentially associated to Brugada Syndrome. These include: 4 genetic variations in AKAP9 including a de novo genetic variation in 3 positive cases; 5 genetic variations in ANK2 detected in 4 cases; variations in KCNJ2 together with CASQ2 in 1 case; genetic variations in RYR2, including a de novo genetic variation and desmosomal proteins encoding genes including DSG2, DSP and JUP, detected in 3 of the cases. Larger gene panels or whole exome sequencing should be considered to identify novel genes associated to Brugada Syndrome. However, application of approaches such as whole exome sequencing would difficult the interpretation for clinical purposes due to the large amount of data generated. The identification of these genetic variants opens new perspectives on the implications of genetic background in the arrhythmogenic substrate for research purposes.As a paradigm for other arrhythmogenic diseases and for unexplained sudden death, our data show that clinical genetic diagnosis is justified in a family perspective for confirmation of genetic causality. In the era of personalized medicine using high-throughput tools, clinical decision-making is increasingly complex.

In 1992 a new syndrome consisting of syncopal episodes and/or sudden death in patients with a structurally normal heart and a characteristic electrocardiogram (ECG) with a pattern of right bundle branch block with an ST segment elevation in leads V1 to V3 was described 1 In 1998 the poor prognosis of patients with the syndrome not receiving an implantable defibrillator was reported 2,3 In 1998 the genetic nature of the disease and its assotiation to a mutation in the cardiac sodium channel gene was described 4 . Because the diagnosis is easily made by means of the ECG, an increasing number of patients with the ECG pattern are being identified worldwide. In this article we will review our present knowledge concerning patients with the classical ECG pattern of the disease. In the Brugada syndrome, the diagnosis is based on the history of aborted sudden death with the typical electrocardiographic pattern of ST segment elevation in leads V1-V3, with or without right bundle branch block 1 ( Fig. 1). In some cases, however, the diagnosis is different because some individuals present with an abnormal electrocardiogram but are completely asymptomatic or there is a history of sudden death in the family and the electrocardiographic criteria are observed.

BackgroundAlthough the assessment of diastolic dysfunction (DD) is an integral part of routine cardiologic examinations, little is known about associated electrocardiographic (ECG) changes. Our aim was to investigate a potential role of ECG indices for the recognition of patients with DD.Methods and resultsECG parameters correlating with echocardiographic findings of DD were retrospectively assessed in a derivation group of 172 individuals (83 controls with normal diastolic function, 89 patients with DD) and their diagnostic performance was tested in a validation group of 50 controls and 50 patients. The patient group with a DD Grade 1 and 2 showed longer QTc (422 ± 24 ms and 434 ± 32 ms vs. 409 ± 25ms, pConclusionsA novel electrocardiographic index Tend-P/(PQxAge) demonstrates a high diagnostic accuracy for the diagnosis of DD and yields a substantial added value when combined with the LAESVI.

BackgroundFabry disease (FD) is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (GLA) resulting in the accumulation of globotriaosylsphingosine (Gb3) in a variety of tissues. While GLA deficiency was always considered as the fulcrum of the disease, recent attention shifted towards studying the mechanisms through which Gb3 accumulation in vascular cells leads to endothelial dysfunction and eventually multiorgan failure. In addition to the well-described macrovascular disease, FD is also characterized by abnormalities of microvascular function, which have been demonstrated by measurements of myocardial blood flow and coronary flow reserve. To date, the relative importance of Gb3 accumulation versus GLA deficiency in causing endothelial dysfunction is not fully understood; furthermore, its differential effects on cardiac micro- and macrovascular endothelial cells are not known.Methods and resultsIn order to assess the effects of Gb3 accumulation versus GLA deficiency, human macro- and microvascular cardiac endothelial cells (ECs) were incubated with Gb3 or silenced by siRNA to GLA. Gb3 loading caused deregulation of several key endothelial pathways such as eNOS, iNOS, COX-1 and COX-2, while GLA silencing showed no effects. Cardiac microvascular ECs showed a greater susceptibility to Gb3 loading as compared to macrovascular ECs.ConclusionsDeregulation of key endothelial pathways as observed in FD vasculopathy is likely caused by intracellular Gb3 accumulation rather than deficiency of GLA. Human microvascular ECs, as opposed to macrovascular ECs, seem to be affected earlier and more severely by Gb3 accumulation and this notion may prove fundamental for future progresses in early diagnosis and management of FD patients.

Descritas as características anátomo-clinicas da displasia ventricular direita arritmogênica e discutidos os métodos diagnósticos e terapêuticos, é apresentada a Série de Maastricht constituída de 14 pacientes, com os dados clínicos, diagnósticos e terapêuticos num tempo médio de 4.2 anos de acompanhamento.