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

Background And 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: 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 centers, between 1980 and 2022.

Independent External Evaluation of Pediatric Hypertrophic Cardiomyopathy Risk Scores in Predicting Severe Ventricular Arrhythmias.

Background: Sudden cardiac death is the most common cause of death in childhood hypertrophic cardiomyopathy (HCM). Recently, 2 risk scores have been developed to estimate the 5-year risk of sudden cardiac death. We aimed to assess their respective performances in an independent cohort.

Age-Related Ventricular Tachycardia Substrate Characteristics for Repaired Tetralogy of Fallot Before Transcatheter Pulmonary Valve Placement.

Background: Ventricular tachycardia (VT) substrate characteristics before transcatheter pulmonary valve replacement (TPVR) in repaired tetralogy of Fallot (rTOF) are unknown.

Objectives: In this study, the authors sought to evaluate substrates for sustained monomorphic VT before TPVR in rTOF.

Methods: Retrospective (2017 to 2021) and prospective (commencing 2021) rTOF patients with native right ventricular outflow tract referred for electrophysiology study (EPS) before TPVR were included.

External Validation of a Risk Score Model for Predicting Major Clinical Events in Adults After Atrial Switch.

Background: A risk model has been proposed to provide a patient individualized estimation of risk for major clinical events (heart failure events, ventricular arrhythmia, all-cause mortality) in patients with transposition of the great arteries and atrial switch surgery. We aimed to externally validate the model.

Methods And Results: A retrospective, multicentric, longitudinal cohort of 417 patients with transposition of the great arteries (median age, 24 years at baseline [interquartile range, 18-30]; 63% men) independent of the model development and internal validation cohort was studied.

First in man right atrial appendage implantation of a Micra leadless pacemaker.

Introduction: Pacemaker implantation can be challenging in patients with congenital heart disease.

Methods And Results: In a patient with Ebstein disease and symptomatic sinus node dysfunction, despite multiple attempts, the Micra® pacemaker could not be implanted in the severely dilated right ventricle. In that context, and after iodine injection to confirm the appropriate location, the Micra® pacemaker was successfully implanted in the right appendage at the first attempt.

Antiplatelet and Anti-Coagulation Therapy for Left-Sided Catheter Ablations: What Is beyond Atrial Fibrillation?

International guidelines on the use of anti-thrombotic therapies in left-sided ablations other than atrial fibrillation (AF) are lacking. The data regarding antiplatelet or anticoagulation strategies after catheter ablation (CA) procedures mainly derive from AF, whereas for the other arrhythmic substrates, the anti-thrombotic approach remains unclear. This survey aims to explore the current practices regarding antithrombotic management before, during, and after left-sided endocardial ablation, not including atrial fibrillation (AF), in patients without other indications for anti-thrombotic therapy.

Catheter ablation of atrial tachyarrhythmias in patients with atrioventricular septal defect.

Aims: The incidence of atrial tachyarrhythmias is high in patients with atrioventricular septal defect (AVSD). No specific data on catheter ablation have been reported so far in this population. We aimed to describe the main mechanisms of atrial tachyarrhythmias in patients with AVSD and to analyse outcomes after catheter ablation.

Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part I: JACC State-of-the-Art Review.

Patients with congenital heart disease associated with a higher risk for ventricular arrhythmias (VA) and sudden cardiac death (SCD) can be divided conceptually into those with discrete mechanisms for reentrant monomorphic ventricular tachycardia (VT) (Group A) and those with more diffuse substrates (Group B). Part I of this review addresses Group A lesions, which predominantly consist of tetralogy of Fallot and related variants. Well-defined anatomic isthmuses for reentrant monomorphic VT are interposed between surgical scars and the pulmonary or tricuspid annulus.

Sinus rhythm QRS morphology reflects right ventricular activation and anatomical ventricular tachycardia isthmus conduction in repaired tetralogy of Fallot.

Background: Patients with repaired tetralogy of Fallot (TOF) are at risk for ventricular tachycardia (VT) related to well-described anatomical isthmuses.

Objective: The purpose of this study was to explore QRS morphology as an indicator of anatomical isthmus conduction.

Methods: Patients with repaired TOF and complete right bundle branch block referred for transcatheter pulmonary valve replacement (PVR) or presenting with sustained VT underwent comprehensive 3-dimensional mapping in sinus rhythm.

Rationale and Design of the Multicenter Catheter Ablation of Ventricular Tachycardia Before Transcatheter Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Study.

Patients with repaired tetralogy of Fallot are at elevated risk for ventricular arrhythmia and sudden cardiac death. Over the past decade, the pathogenesis and natural history of ventricular tachycardia has become increasingly understood, and catheter ablation has emerged as an effective treatment modality. Concurrently, there has been great progress in the development of a versatile array of transcatheter valves that can be placed in the native right ventricular outflow tract for the treatment of long-standing pulmonary regurgitation.

Ventricular arrhythmia in congenital heart diseases with a systemic right ventricle.

Congenital heart disease (CHD) often involves the systemic right ventricle (SRV), which is the morphological right ventricle that supports systemic circulation. SRV patients are at a higher risk of sudden cardiac death (SCD) than other adult CHD patients and continues to be a significant cause of death in this aging population. However, the pathophysiology of ventricular arrhythmias in SRV is still not fully understood, and there may be differences between subtypes of CHD.

Management for atrial arrhythmias in adults with complex congenital heart disease.

Introduction: The prevalence of congenital heart disease (CHD) is steadily increasing among adults. Atrial arrhythmias are frequent late complications and are associated with substantial morbidity.

Areas Covered: We discuss key considerations regarding management strategies for atrial arrhythmias in common forms of CHD and offer future perspectives.

Sudden cardiac death after heart transplantation: a population-based study.

Aims: The epidemiology of sudden cardiac death (SCD) after heart transplantation (HTx) remains imprecisely described. We aimed to assess the incidence and determinants of SCD in a large cohort of HTx recipients, compared with the general population.

Methods And Results: Consecutive HTx recipients (n = 1246, 2 centres) transplanted between 2004 and 2016 were included.

Systematic Electrophysiological Study Prior to Pulmonary Valve Replacement in Tetralogy of Fallot: A Prospective Multicenter Study.

Background: Ventricular arrhythmias and sudden death are recognized complications in tetralogy of Fallot. Electrophysiological studies (EPS) before pulmonary valve replacement (PVR), the most common reintervention in tetralogy of Fallot, could potentially inform therapy to improve arrhythmic outcomes.

Methods: A prospective multicenter study was conducted to systematically assess EPS with programmed ventricular stimulation in patients with tetralogy of Fallot referred for PVR from January 2020 to December 2021.