BACKGROUND AND AIMS:Current evidence on catheter ablation for patients with congenital heart disease (CHD) is derived from small, retrospective studies. This study aims to provide insights from a nationwide contemporary registry. METHODS:This prospective study included all CHD patients referred for catheter ablation from 2020 to July 2024 across 28 French centres. The primary outcome was the rate of per-procedural acute success. Secondary outcomes included complications as well as freedom from arrhythmia recurrence. RESULTS:A total of 1135 consecutive catheter ablation procedures were performed in 998 patients (mean age 46.1 ± 16 years, 55.5% male). The main primary clinical arrhythmias targeted were atrial flutter/tachycardia in 677 (59.6%), atrial fibrillation in 195 (17.2%), ventricular arrhythmia in 188 (16.6%), and atrioventricular reentrant tachycardia in 38 (3.3%), with significant variations in patterns observed based on the underlying substrate. Clinical arrhythmia was successfully ablated in 1071 patients (94.4%). The mean number of arrhythmias targeted per procedure was 1.5 ± 0.7, with overall acute success rates exceeding 90% for all arrhythmias except for ventricular arrhythmias (86.7%). Acute complication occurred in 43 procedures (3.8%), including 1 (0.1%) death. The overall 1- and 2-year recurrence-free rates were 77.3% (95% confidence interval 74.2%-80.4%) and 68.4% (95% confidence interval 64.7%-72.3%), respectively. Significant variations in recurrence rates were noted based on the type of arrhythmia and the underlying CHD. CONCLUSIONS:Catheter ablation in patients with CHD demonstrates highly favourable acute outcomes and a low complication rate. Recurrence rates during follow-up vary depending on the targeted arrhythmia and the underlying CHD. These findings should be considered in the benefit-risk assessment.

Background Recurrence rates of atrial arrhythmias after catheter ablation in patients with congenital heart disease (CHD) remain substantial. Given the high prevalence of peritricuspid flutter, systematic cavotricuspid isthmus (CTI) ablation may represent a rational preventive strategy. Objectives The objective of this study was to assess the impact of empirical CTI ablation in CHD patients. Methods This prospective nationwide study included all CHD patients referred for a first catheter ablation of an atrial arrhythmia between 2020 and 2024. Empirical ablation comprised probabilistic (noninducible arrhythmia with prior documentation) and prophylactic (no prior documentation) ablation. The primary endpoint was freedom from any atrial arrhythmia recurrence. Secondary endpoints included arrhythmia-related hospitalizations, redo ablations, and complications. Results Among 575 patients undergoing a first catheter ablation for atrial arrhythmia, 345 (60.0%) had CTI-dependent flutter. The remaining 230 patients formed the study population (mean age: 48.2 ± 16.4 years; 50.9% male). CHD complexity was predominantly moderate (57.4%). Targeted arrhythmias were atrial macro-re-entry (53.0%), atrial fibrillation (47.0%), and focal atrial tachycardia (17.4%), with multiple arrhythmias in 16.5%. Empirical CTI ablation was performed in 71 patients (30.9%) and was associated with significantly higher freedom from any atrial arrhythmia at 1 year (78.1% vs 65.6%, P = 0.010) and 2 years (71.2% vs 56.1%, P = 0.025), remaining independently associated with lower risk of recurrence after multivariable adjustment (HR: 0.47, 95% CI: 0.24-0.92, P = 0.027). This benefit was primarily observed in patients without atrial fibrillation history (interaction P = 0.005). Arrhythmia-related hospitalization was also significantly reduced (14.1% vs 25.8%, P = 0.048). Conclusions Empirical CTI ablation is associated with improved freedom from atrial arrhythmia and reduced rehospitalization in CHD patients.

Introduction Percutaneous closure of ostium secundum atrial septal defect (ASD) has become the standard of care for this condition. However the occurrence of supraventricular arrhythmias remain insufficiently studied. We conducted this study to identify predictors of long-term supraventricular arrhythmias following percutaneous ASD closure. Methods We performed a retrospective single-center study including patients who underwent percutaneous ostium secundum ASD closure at Marie Lannelongue Hospital between 1998 and 2021. Procedural and long-term follow-up data were systematically collected. Patients with a prior history of supraventricular arrhythmia were excluded from the main analysis. A comparative analysis was then performed between patients who developed new-onset supraventricular arrhythmia during long-term follow-up and those who did not. Results Among the 1,495 patients without pre-procedural atrial arrhythmia, 98 (6.5%) developed atrial arrhythmia during long-term follow-up (median follow-up: 10.0 years). In univariate analysis, patients who developed post-procedural arrhythmias were significantly older (48.5 vs. 18.0 years, p<0.001), more frequently had a body mass index≥30kg/m2 (p=0.003), and had higher CHA2DS2-VASc scores (p<0.001). On baseline electrocardiography, complete right bundle branch block (p=0.04) and atrial premature beats (p=0.009) were more frequent. Echocardiographic features associated with arrhythmia included interatrial septal aneurysm (p=0.008) and left ventricular dysfunction (p=0.01). Hemodynamic data showed significantly higher pulmonary artery pressures (p<0.001), and larger device size was also associated with arrhythmia occurrence (p<0.001). During long-term follow-up, patients who developed atrial arrhythmias experienced significantly higher rates of stroke, heart failure, pacemaker implantation, and all-cause mortality (all p<0.001). Multivariate analysis is currently ongoing, and a predictive risk score for long-term supraventricular arrhythmia after ASD closure is under development. Conclusion New-onset atrial arrhythmia represents a clinically significant source of morbidity after percutaneous ASD closure. Identification of predictive factors and development of a dedicated risk score may allow more targeted long-term rhythm monitoring in these patients.

Introduction Conduction System Pacing (CSP) with his bundle or left bundle branch stimulation is an attractive emerging pacing mode associated with better haemodynamic outcomes in general population. Preliminary datas are encouraging in congenital heart disease (CHD). Method In this study with prospectively evaluate feasibility, safety and efficacy of CSP in consecutive CHD with indication of ventricular pacing. Results Between January 2022 and December 2024, 14 CHD were referred for pacing: atrioventricular canal defect (3), atrial septal defect (2), tetralogy of Fallot (2), TGA with a trial switch (2), ccTGA (1), congenital aortic stenosis (3), Ebstein anomaly (1). Indications for ventricular stimulation were: complete AV block (6), his bundle ablation for supraventricular tachycardias (3), pacing lead dysfunction (3), ventricular resynchronization (2). Mean follow-up was 11±8 months. CSP was achieved in 100% with good pacing parameters, with 1 acute complication (local haematoma). Systemic ejection fraction after pacing was stable in 6 (42%), increased in 8 (56%). Conclusion CSP in feasible in a was majority of CHD with endocavitary ventricular access. It avoids adverse haemodynamic effect of ventricular pacing with a potential effect of ventricular resynchronization with increase haemodynamics. CSP should by offered in CHD patients with indication for ventricular pacing.

In the presence of recurrent sustained monomorphic ventricular tachycardia (VT) catheter ablation may be an option in congenital heart disease (CHD). However, the unusual substrate and anatomy limit this option to few expert electrophysiologists. We sought to investigate whether VT isthmus could be identified on 3D imaging substrate reconstruction from cardiac computed tomography scan (CT-scan) and/or magnetic resonance imaging (MRI) before ablation in CHD. Forty consecutives patients with cardiac CT scan or MRI referred to VT ablation in 4 congenital electrophysiology centres were studied. Three electrophysiologists (EP) blinded from the ablation procedures and to each other's annotated ablation targets on 3d imaging substrate reconstruction (Figure 1). Once this was done, annotations were compared to each other and to ablation target on the electro-anatomical map performed during the procedure. A total of 40 patients ( 27 male, 37 ±12 yo) with VT ablation in the setting of repaired tetralogy of Fallot (20), transposition of the great arteries (8), pulmonary atresia with VSD (3) or other CHD (9) were studied. In 5 patients, no VT isthmus was identified during the procedure but the substrate targeted was identified on imaging. In 30/40, the VT isthmus was identified on imaging. In the remaining 5, the VT isthmus was not identified on imaging. In one no scar was identified. In 2 patients, all 3 EP agreed on a strategy which did not include the isthmus and in 2 other patients, 2 EP agreed on a strategy not including the isthmus. All 3 EP identified the same target in 28 patients, 2/3 EP in 10, 1/3 identify a target in 1 patient and no target was identified by any of the 3 EP in one patient. 3D substrate reconstruction identifies VT isthmus with certainty in 75% of patients referred for VT ablation in the setting of CHD. In 87.5%, it identifies substrate ablated during the procedure.rToF patient with cMRI Double Outlet RV pt with CT scan

BACKGROUND:Sudden death and ventricular arrhythmias (VAs) remain a significant concern among patients with congenital heart disease (CHD). Although catheter ablation techniques have improved dramatically over the last decade, current evidence in this specific population is primarily derived from small retrospectvie studies. OBJECTIVES:The aim of this study was to describe the burden and characteristics of VAs targeted by catheter ablation in CHDs, as well as associated outcomes and emerging preventive ablative strategies. METHODS:This prospective nationwide study included all patients with CHD referred for catheter ablation of a VA from 2020 to 2024 in France. The primary outcome was the rate of per-procedural acute success. Secondary outcomes included complications as well as freedom from arrhythmia recurrence. RESULTS:Among a total of 1,192 consecutive catheter ablation procedures, 210 (17.6%) VA catheter ablations were performed in 190 patients (mean age 43.8 ± 15.5 years; 63.8% male): ventricular tachycardia (VT) was targeted in 164 (78.1%) procedures and premature ventricular complex in 53 (25.2%) (both VT and premature ventricular complex were targeted in 7). Fourteen (6.7%) patients had a simple CHD, 161 (76.7%) a moderate CHD, and 35 (16.7%) a complex CHD. In patients with tetralogy of Fallot (n = 126), catheter ablation was performed without clinically documented VA in 46 (36.5%), mainly before transcatheter or surgical intervention. Overall, the clinical arrhythmia was successfully ablated in 182 (86.7%) patients. An acute complication was reported in 6 (2.9%) procedures, with no related death. The overall 1- and 2-year rates of freedom from recurrence were 81.5% (95% CI: 75.3%-88.4%) and 78.2% (95% CI: 71.2%-85.8%), respectively. The presence of anatomical isthmuses related to prior cardiac surgeries was associated with lower recurrence rates (HR: 0.30; 95% CI: 0.14-0.64; P < 0.001). CONCLUSIONS:VAs represent approximately 20% of catheter ablation procedures performed in patients with CHD. This large cohort provides key insights into the effectiveness of catheter ablation and the main mechanisms of VAs in patients with CHD. The significant differences in outcomes reported depending on underlying substrate are important to consider to inform the benefit/risk assessment.

BACKGROUND:Tetralogy of Fallot (TOF), the most common cyanogenic congenital heart disease at birth, often leads to later complications such as pulmonary regurgitation (PR), right ventricular failure, and atrial or ventricular arrhythmias after complete surgical repair. Percutaneous pulmonary valve implantation (PPVI) is an alternative to surgery to treat a right-ventricular outflow tract dysfunction. CASE SUMMARY:We present here an atypical case of an emergency PPVI in a 69-year-old TOF patient in refractory cardiogenic shock with severe PR decompensated by new-onset rapid atrial fibrillation. DISCUSSION:Recent data show that PPVI is usually carried out on an elective basis, with procedures rarely performed in emergencies.

Abstract Background Ablation of persistent atrial fibrillation (AF) beyond pulmonary vein isolation (PVI) remains challenging, despite increasing numbers of eligible patients. Previous studies suggest a possible role for non-PVI lesions targeting atrial drivers, but their identification can be difficult and subject to inter-operator variability. Purpose We investigated feasibility for an electrogram-based artificial intelligence software system to assist in the identification of left or right atrial dispersion sites as ablation targets for persistent AF using the penta-spline multi-electrode pulsed-field ablation (PFA) catheter, a recent technique which is more conventionally employed for PVI. Methods This is a single-centre retrospective descriptive study of a cohort of patients with persistent AF, who underwent their first-time ablation from May to November 2023 using the Farawave PFA catheter (Boston Scientific) and the VX1 system (Volta Medical). This system relies on off-line pre-training of multiparametric machine learning algorithms on a database of annotated AF intracardiac electrograms. It connects to cardiac electrophysiology recording systems via a custom data cable to receive analogue data and digitize them through an integrated converter. Its algorithms analyse signals in the cardiac chamber of interest to adjudicate the local presence or absence of dispersion and provides relevant visual cues to operators in order to target energy delivery. Results We identified 16 patients who underwent PFA for persistent AF (long-standing in 5/16, 31.3%), including PVI and further ablation guided by VX1. See table 1 for baseline population characteristics and table 2 for procedural characteristics. All underwent ablation with uninterrupted anticoagulation, general anaesthesia, use of the Rhythmia electroanatomic mapping system and its Orion mapping catheter (Boston Scientific) to feed signals into the VX1 system. VX1-guided electrogram localization and ablation mostly targeted the anterior region of the left atrium (16 segments), followed by its septal region (6 segments) and its lateral region (4 segments); the median number of applications per procedure was 88 [IQR 68-108], including a median of 76 [61-85] in the left atrium and 12 [0-15] applications in the right atrium. The median procedure duration was 123 [114-137] minutes; the median fluoroscopy time was 32 [26-40] minutes and the median radiation dose was 12.5 Gy∙cm2 [10.0-25.8]. Sinus rhythm was restored either during ablation or by electrical cardioversion (see Table 2). Two complications occurred one femoral arteriovenous fistula requiring stenting and one tamponade requiring drainage. At a three-month follow-up, 87.5% did not present AF recurrences. Conclusion Real-time detection and annotation of electrograms to track dispersion using AI-assisted technology to support PFA with a multipolar catheter is feasible. The interest of the approach should be validated by further studies.Table 1.Table 2.

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. Although these events are rare, predicting them is challenging. This review discusses contemporary strategies for assessing and preventing the risk of ventricular arrhythmias in SRV patients. Several risk factors have been identified to be associated with ventricular arrhythmias in patients with SRV. A recent risk stratification model combines independently associated factors into a risk score, and subpulmonary left ventricle dysfunction is emerging as a critical factor in risk assessment. Cardiac magnetic resonance imaging, biomarkers, and genetic data may refine the ability to predict ventricular arrhythmias in SRV. However, the question of whether implantable cardioverter-defibrillators (ICDs) should be used as a preventive measure in this cohort remains unanswered. Multicenter studies are needed to evaluate risk models and ICD use in this aging population. Given that ICDs have drawbacks, such as a high rate of inappropriate shocks and late lead-related complications, shared clinical decision-making is crucial when considering their use. The review emphasizes the need for further research in this area to improve the identification of patients at risk of clinical ventricular arrhythmias and to develop effective prevention strategies.

Background Robotic magnetic navigation (RMN) has emerged as a potential solution to overcome challenges associated with catheter ablation of arrhythmias in patients with congenital heart disease (CHD).Objectives To assess safety and efficacy of RMNguided catheter ablation in patients with CHD.Design and Methods A systematic review and pooled analysis was conducted on patients with CHD who underwent RMNguided catheter ablation. Random effects models were used to generate pooled estimates with the inverse variance method used for weighting studies.Results Twentyfour nonoverlapping records included 167 patients with CHD, mean age 36.5 years, 44.6% female. Type of CHD was simple in 27 (16.2%), moderate in 32 (19.2%), and complex in 106 (63.5%). A total of 202 procedures targeted 260 arrhythmias, the most common being macroreentrant atrial circuits. The mean procedural duration was 207.5 minutes, with a mean fluoroscopy time of 12.1 minutes. The pooled acute success rate was 89.2% [95% CI (77.8%, 97.4%)]. Freedom from arrhythmia recurrence was 84.5% [95% CI (72.5%, 94.0%)] over a mean follow-up of 24.3 months. The procedural complication rate was 3.5% with no complication attributable to RMN technology.Conclusion RMN-guided ablation appears to be safe and effective across a variety of arrhythmia substrates and types of CHD.

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.Expert opinion An appreciation of the types of atrial arrhythmias encountered in patients with diverse forms of CHD, combined with the growing clinical and research experience, appears to be yielding favorable results, whereas little progress has been made on the antiarrhythmic drug front, indications for anticoagulation have considerably evolved. Advances in interventional techniques have propelled catheter ablation to the forefront to treat a variety of atrial arrhythmias in patients with complex CHD. Nevertheless, much work remains to be done to elucidate underlying pathophysiology, triggers, and critical substrates that predispose patients with specific CHD malformations to develop atrial arrhythmias. Future advances could allow for the implementation of individualized, possibly preemptive, approaches to arrhythmia management. With the prevalence of atrial fibrillation on the rise in the aging population with CHD, concerted efforts must be directed toward optimizing patient selection for catheter ablation as well as refining procedural aspects to safely and more effectively improve long-term outcomes.

Arrhythmias are a common complication associated with tetralogy of Fallot (ToF), one of the most prevalent forms of congenital heart disease. As illustrated by this case-based review, various forms of arrhythmias can be encountered across the lifespan of patients with ToF, from infancy to older adulthood. These include atrioventricular block, junctional ectopic tachycardia, and atrial and ventricular arrhythmias. Arrhythmias have important implications on the health and quality of life of patients with ToF and require treatment by caregivers with dedicated expertise. The choice of pharmacologic and/or interventional therapies to alleviate symptoms, avoid complications, and mitigate risks depends in part on the type, severity, and frequency of the arrhythmia, as well as on the particularities of individual clinical scenarios. Preventing, monitoring for, and managing arrhythmias are an integral component of the care of patients with ToF throughout their lifespan that is critical to optimizing health outcomes.

Long-term follow-up data of left atrial appendage (LAA) occlusion in patients with atrial fibrillation (AF) are sparse. To address these data gaps, we analysed the 4-year outcomes of AF patients following LAA occlusion. The was a retrospective cohort study of high-risk patients with AF who underwent successful implantation of the Amulet device at our center between 2014 and 2017. Study endpoints were the rate of stroke, major bleeding and all-cause mortality. We included 71 patients (35.2% females) with a median age of 78 (IQR 73–82) years. Over a median follow-up period of 46 (IQR 19–56) months, the annual rate of ischemic stroke was 1.06 events/100 patient-years (95% CI 0–2.35), hemorrhagic stroke was 1.06 events/100 patient-years (95% CI 0–2.35) and major extracranial bleeding that required unplanned hospital admission was 1.84 events/100 patient-years (95% CI 0.25–3.43). A total of 28 (39.4%) patients died during this period with an annual mortality rate of 10.29 events/100 patient-years (95% CI 7.25–13.32). Our experience suggests that LAA occlusion using the Amulet device appears to be associated with a low risk of ischemic stroke in high-risk AF patients who are deemed unsuitable for oral anticoagulation; however, these patients have a high rate of mortality over the medium to long-term follow-up, and an ongoing significant risk of bleeding and thrombotic events.

Do patients need to adapt to technology improvements? Serge Boveda, Serge Boveda Heart Rhythm Department, Clinique Pasteur, 45, Avenue de Lombez, Toulouse, FranceUniversitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium Corresponding author. Tel: +33 5 62 21 16 45; fax: +33 5 62 21 16 41. E-mail address: sboveda@clinique-pasteur.com https://orcid.org/0000-0002-8425-3544 Search for other works by this author on: Oxford Academic PubMed Google Scholar Stefano Bartoletti Stefano Bartoletti Heart Rhythm Department, Clinique Pasteur, 45, Avenue de Lombez, Toulouse, FranceLiverpool Heart and Chest Hospital, Liverpool, UK Search for other works by this author on: Oxford Academic PubMed Google Scholar EP Europace, Volume 21, Issue 11, November 2019, Pages 1605–1606, https://doi.org/10.1093/europace/euz252 Published: 24 September 2019