The Clinical Significance of Atrial Fibrillation in Non-High-Risk Brugada Syndrome: The BruFib Study.

Background: Atrial fibrillation (AF) occurs in up to 20% of patients with Brugada syndrome (BrS), yet its risk factors and prognostic implications remain uncertain.

Objectives: This study sought to identify risk factors for AF in patients with non-high-risk BrS and to evaluate the impact of AF on ventricular arrhythmias (VAs), sick sinus syndrome (SSS), and stroke in non-high-risk BrS.

Methods: This was a multicenter, retrospective study conducted across 20 international centers.

Phenotypic spectrum of cardiac conduction disturbance and cardiomyopathy linked to titin canonical splice-site variants.

Aims: Truncating variations in the titin gene (TTNtv) are the most common genetic cause of dilated cardiomyopathy (DCM) and have been implicated in various arrhythmic and heart failure phenotypes. Nonetheless, predicting the pathogenicity of a distinct subtype of TTNtv, canonical splice-site variations (TTNcsv), remains challenging. Furthermore, the precise transcriptional and phenotypic consequences associated with TTNcsv remain unclear.

Pain after subcutaneous implantable cardioverter-defibrillator implantation: A secondary analysis of the PRAETORIAN-DFT trial.

Background: The subcutaneous implantable cardioverter-defibrillator (S-ICD) overcomes transvenous lead-related complications. Its extrathoracic design results in a generator twice the size of transvenous ICDs.

Objective: We investigated pain after S-ICD implantation and explore predictors for severe pain.

Impact of Prior Exercise Practice on Disease Characteristics in Desmoplakin-Related Arrhythmogenic Left Ventricular Cardiomyopathy.

Background: The harmful effects of high-intensity (HI) and long duration (LD) exercise have been reported in arrhythmogenic right ventricular cardiomyopathy, including in asymptomatic genetic variant carriers. However, the impact of exercise remains unclear in desmoplakin (DSP) gene variant carriers at risk of arrhythmogenic left ventricular cardiomyopathy (ALVC).

Objectives: The goal of this study was to assess the relationship between HI and LD exercise and the risk of developing an ALVC phenotype and its severity in DSP variant carriers.

Defibrillation Testing During Implantation of Subcutaneous Implantable Cardioverter Defibrillators.

Background: Defibrillation testing (DT) remains recommended during subcutaneous implantable cardioverter defibrillator (S-ICD) implantation due to limited supporting evidence.

Objectives: The objective of this study was to evaluate the long-term impact of DT during S-ICD implantation.

Methods: The HONEST (coHOrte fraNcaise des dEfibrillateurs Sous cuTanés) study is a nationwide, ongoing observational study, including all S-ICD recipients in France (2012-2019).

S-ICD Implantation Following TV-ICD: Insights Into Patients With Infections and Abandoned Leads-the HONEST Cohort.

Background: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) can be a viable option for patients with transvenous ICDs experiencing complications (rescue S-ICD).

Objectives: This study sought to evaluate the outcomes of rescue S-ICD implantation using data from the HONEST French nationwide S-ICD cohort.

Methods: All rescue S-ICD patients were identified.

The diagnostic role of pharmacological provocation testing in cardiac electrophysiology: a clinical consensus statement of the European Heart Rhythm Association and the European Association of Percutaneous Cardiovascular Interventions (EAPCI) of the ESC, the ESC Working Group on Cardiovascular Pharmacotherapy, the Association of European Paediatric and Congenital Cardiology (AEPC), the Paediatric & Congenital Electrophysiology Society (PACES), the Heart Rhythm Society (HRS), the Asia Pacific Heart Rhythm Society (APHRS), and the Latin American Heart Rhythm Society (LAHRS).

The pharmacological provocation test is a pivotal tool in cardiac electrophysiology for the diagnosis of potential causes of sudden cardiac death, sudden cardiac arrest (SCA), arrhythmias, symptoms, or ECG abnormalities. The 2022 European Society of Cardiology Guidelines for the Treatment of Ventricular Arrhythmias and Prevention of Sudden Cardiac Death offered guidance on provocation testing but did not describe the indications and requirements in depth. This clinical consensus statement, led by the European Heart Rhythm Association and approved by major international stakeholders, aims to advise the general cardiologist and the arrhythmia expert who to test and when, where, and how to do it.

Late outcomes of congenital and childhood non-immune, isolated atrioventricular block: a French nationwide retrospective cohort study.

Aims: The natural history of congenital or childhood non-immune, isolated atrioventricular block (AVB) is poorly defined. We aimed at clarifying its long-term outcomes.

Methods And Results: We retrospectively studied 385 children with isolated, non-immune AVB diagnosed from in utero or up to 18 years of age, at 29 French medical centres, between 1980 and 2022.

Temporal trends in population characteristics and type of device among primary prevention implantable cardioverter defibrillator recipients: The DAI-PP programme.

Background: Patient characteristics, technology and clinical practice surrounding primary prevention implantable cardioverter defibrillators have evolved continuously over time.

Aim: To explore the temporal changes in patient characteristics, pharmacological therapy and device types among implantable cardioverter defibrillator recipients implanted for the primary prevention of sudden cardiac death over the last two decades in France.

Methods: Characteristics of participants and type of device from the retrospective DAI-PP Pilot Study (2002-2012) were compared with those from the ongoing prospective DAI-PP Consortium (2018 onwards).

International Multicenter Cohort Study on Beta-Blocker-Free Treatment Strategies for Catecholaminergic Polymorphic Ventricular Tachycardia Patients.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, potentially life-threatening genetic heart disease. Nonselective beta-blockers (BBs) are highly effective in reducing CPVT-triggered arrhythmic events. However, some patients suffer from unacceptable BB side effects and might require strategies without a BB.

Novel risk predictor of arrhythmias for patients with potassium channel-related congenital long QT syndrome.

Background: Congenital long QT syndrome (LQTS) is characterized by delayed ventricular repolarization, predisposing to potentially lethal ventricular arrhythmias. The variability in disease severity among patients remains largely unexplored, underscoring the limitations of current risk stratification methods.

Objective: We aimed to evaluate the potential utility of electrocardiographic markers from the exercise stress test (EST) in identifying patients with high-risk LQTS.

Long-term prognosis of patients with an SCN5A loss-of-function variant and progressive cardiac conduction disorder or Brugada syndrome.

Background: The long-term prognosis of patients with a loss-of-function variant in the cardiac sodium channel gene SCN5A is unknown.

Objective: This study aimed to evaluate the long-term arrhythmic risk in patients with an SCN5A loss-of-function variant to identify predictors of arrhythmic events.

Methods: Probands and family members with (likely) pathogenic SCN5A loss-of-function variants were retrospectively included.

Isolated prolapse of the posterior mitral valve leaflet: phenotypic refinement, heritability and genetic etiology.

Background: Isolated posterior leaflet mitral valve prolapse (PostMVP), a common form of MVP, often referred as fibroelastic deficiency, is considered a degenerative disease. PostMVP patients are usually asymptomatic and often undiagnosed until chordal rupture. The present study aims to characterize familial PostMVP phenotype and familial recurrence, its genetic background, and the pathophysiological processes involved.

Description of the Two-Dimensional Layer-Specific Strain Echocardiography Phenotype of Arrhythmogenic Left Ventricular Cardiomyopathy.

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is characterized by fibrofatty myocardial replacement demonstrated on cardiac magnetic resonance by late gadolinium enhancement (LGE) mainly involving the subepicardium. The aims of this study were to describe the layer-specific strain (LSS) echocardiography phenotype of ALVC and to compare it with LGE features.

Methods: All consecutive ALVC pathogenic genetic variant carriers and noncarrier relatives were separated into four prespecified groups (overt ALVC [group 1], isolated LGE [group 2], pathogenic genetic variant carrier without ALVC phenotype [group 3], and no genetic variant carrier [group 4]) and studied accordingly using cardiac magnetic resonance and LSS echocardiography.

Personalized screening before subcutaneous cardioverter-defibrillator implantation: Usefulness and outcomes in clinical practice-the S-ICD screening SIS prospective study.

Background: Electrocardiographic screening before subcutaneous implantable cardioverter-defibrillator (S-ICD) implantation is unsuccessful in around 10% of cases. A personalized screening method, by slightly moving the electrodes, to obtain a better R/T ratio has been described to overcome traditional screening failure.

Objective: The objectives of the SIS study were to assess to what extent a personalized screening method improves eligibility for S-ICD implantation and to evaluate the inappropriate shock rate after such screening success.

Brugada syndrome in Japan and Europe: a genome-wide association study reveals shared genetic architecture and new risk loci.

Background And Aims: Brugada syndrome (BrS) is an inherited arrhythmia with a higher disease prevalence and more lethal arrhythmic events in Asians than in Europeans. Genome-wide association studies (GWAS) have revealed its polygenic architecture mainly in European populations. The aim of this study was to identify novel BrS-associated loci and to compare allelic effects across ancestries.

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there is limited data on the outcomes of ICD use in children.

When to ablate in Brugada and early repolarization syndromes.

Introduction: Mapping advances have expanded both the feasibility and benefits of ablation as a therapeutic approach, including in the treatment of two heart conditions that contribute to sudden cardiac death in young people: Brugada syndrome (BrS) and early repolarization syndrome (ERS). Although these conditions share a number of similarities, debates persist regarding the underlying pathophysiology and origin of the ventricular arrhythmias associated with them.

Areas Covered: By synthesizing available data (PubMed), including current recommendations, pathophysiological insights and case reports, patient registries, our aim is to elucidate and establish the nuanced role of radiofrequency ablation (RFA) in therapeutic management.

Insights into adherence to medication and lifestyle recommendations in an international cohort of patients with catecholaminergic polymorphic ventricular tachycardia.

Aims: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), a rare inherited arrhythmia syndrome, arrhythmic events can be prevented by medication and lifestyle recommendations. In patients who experience breakthrough arrhythmic events, non-adherence plays an essential role. We aimed to investigate the incidence and potential reasons for non-adherence to medication and lifestyle recommendations in a large, international cohort of patients with CPVT.

Predictive value of the PRAETORIAN score for defibrillation test success in patients with subcutaneous ICD: A subanalysis of the PRAETORIAN-DFT trial.

Background: The PRAETORIAN score estimates the risk of failure of subcutaneous implantable cardioverter-defibrillator (S-ICD) therapy by using generator and lead positioning on bidirectional chest radiographs. The PRospective randomized compArative trial of subcutanEous implanTable cardiOverter-defibrillatoR ImplANtation with and without DeFibrillation Testing (PRAETORIAN-DFT) investigates whether PRAETORIAN score calculation is noninferior to defibrillation testing (DFT) with regard to first shock efficacy in spontaneous events.

Objective: This prespecified subanalysis assessed the predictive value of the PRAETORIAN score for defibrillation success in induced ventricular arrhythmias.

Pre-emptive treatment of heart failure exacerbations in patients managed with the HeartLogic™ algorithm.

Aims: Heart failure (HF) is a chronic disease affecting 64 million people worldwide and places a severe burden on society because of its mortality, numerous re-hospitalizations and associated costs. HeartLogic™ is an algorithm programmed into implanted devices incorporating several biometric parameters which aims to predict HF episodes. It provides an index which can be monitored remotely, allowing pre-emptive treatment of congestion to prevent acute decompensation.

Clinical Management of Brugada Syndrome: Commentary From the Experts.

Although there is consensus on the management of patients with Brugada Syndrome with high risk for sudden cardiac arrest, asymptomatic or intermediate-risk patients present clinical management challenges. This document explores the management opinions of experts throughout the world for patients with Brugada Syndrome who do not fit guideline recommendations. Four real-world clinical scenarios were presented with commentary from small expert groups for each case.