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The Implantable cardioverter-defibrillator (ICD) has been proven to improve survival in adults with congenital heart disease (ACHD), however it is associated with a high rate of complications. We aimed to quantify the incidence of early (≤3 months) and late (3 months) complications (EC, LC) in ACHD patients implanted with an ICD and identify their clinical predictors.We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General Hospital between 1996 and 2019.Most common diagnoses were Tetralogy of Fallot (32.4%), dextro-transposition of the great arteries (17.9%), congenital corrected transposition of the great arteries (13%). No intraprocedural complications were observed. Median follow up was 3.4 years (0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgments). A total of 56 LC occurred (46% lead failure, 21% infection, 11% prophylactic lead extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LC of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9,p=0.02) and CRTD implant(OR 2.5,p=0.04) were associated with EC. Survival free from LC was 92%, 86%, 65% respectively after 1,5 and 10 years. Presence of legacy leads (hazard ratio 2.9,p=0.006) and subpulmonary ejection fraction (5% increase, HR 1.35,p=0.031) were associated with LC.ACHD patients at risk of SCD continue to benefit from newer device technology. However, these patients-particularly those with greater anatomic and device complexity-remain at increased risk of developing complications over their lifetime. Given the life expectancy of this population, careful consideration needs to be given when a device for primary prevention is being contemplated.

Decrement evoked potentials (EPs) (DeEPs) constitute an accepted method to identify physiological ventricular tachycardia (VT) ablation targets without inducing VT. The feasibility of automated software (SW) in the detection of arrhythmogenic VT substrate has been documented. However, multicenter validation of automated SW and workflow has yet to be characterized. The objective of this study was to describe the functionality of a novel DeEP SW (Biosense Webster, Diamond Bar, CA, USA) and evaluate the independent performance of the automated algorithm using multicenter data. VT ablation cases were performed in the catheterization laboratory and retrospectively analyzed using the DeEP SW. The algorithm indicated and mapped DeEPs by first identifying capture in surface electrocardiograms (ECGs). Once capture was confirmed, the EPs of S1 paces were detected. The algorithm checked for the stability of S1 EPs by comparing the last 3 of the 8 morphologies and attributing standard deviation values. The extra-stimulus EP was then detected by comparing it to the S1 EP. Once detected, the DeEP value was computed from the extra-stimulus and displayed as a sphere on a voltage map. A total of 5,885 DeEP signals were extracted from 21 substrate mapping cases conducted at 3 different centers (in Spain, Canada, and Australia). A gold standard was established from ECGs manually marked by subject experts. Once the algorithm was deployed, 91.6% of S2 algorithm markings coincided with the gold standard, 1.9% were false-positives, and 0.1% were false-negatives. Also, 6.4% were non-specific DeEP detections. In conclusion, the automated DeEP algorithm identifies and displays DeEP points, revealing VT substrates in a multicenter validation study. The automation of identification and mapping display is expected to improve efficiency.

Delivery of electrophysiology (EP) care in developing nations and underserviced populations faces many hurdles, including the lack of local expertise and knowledge creation. The West Indies has experienced a paucity of local EP expertise. The University of Toronto has undertaken a unique collaborative educational effort with the University of the West Indies.We describe the effects of equity, diversity, and inclusion (EDI) in EP training at Toronto General Hospital in Canada by quantifying the impact of training the first female electrophysiologists to practice in Jamaica and Saint Lucia.Data from the ministries of health in Jamaica and Saint Lucia were reviewed. The number of arrhythmia clinic patients seen, EP studies and ablations performed, pacemaker clinic patients seen, and implantable devices, permanent pacemakers (PPMs), and implantable cardioverter-defibrillators (ICDs) implanted were assessed.One hundred one arrhythmia consults were seen by the new electrophysiologist in Jamaica after her return from training in 2020. She has since performed 19 EP studies/catheter ablations at a newly established ablation laboratory. Three cases of left ventricular (LV) dysfunction due to tachy-cardiomyopathy were treated successfully with catheter ablation with immense improvement in LV ejection fraction. Thirteen PPMs, 1 ICD, and 3 LV leads were implanted, after which no early complications were identified. In Saint Lucia, where there is no dedicated electrophysiology laboratory, 2 patients who required catheter ablation for tachycardia-mediated LV dysfunction were identified by the electrophysiologist since her return to the island in 2018. The patients were appropriately referred, resulting in restoration of normal LV function. Six PPMs also were implanted in Saint Lucia. Knowledge translation has been limited by the lack of accessibility to the required devices, catheters, and specialized equipment and accessories, mainly because of their costs.Training the first female electrophysiologists from Jamaica and Saint Lucia led to a quantifiable impact on EP care in both of these Caribbean countries. EDI strategies in EP training programs provide much needed benefits to developing nations, but more support is needed to allow new electrophysiologists to fully utilize their EP training to care for underserviced populations.

Interventions in adults with congenital heart disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigour to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. In addition to tables indexed to evidence, clinical flow diagrams are included for each lesion to facilitate a practical approach to clinical decision-making. Excluded are recommendations for pacemakers, defibrillators, and arrhythmia-directed interventions covered in separate designated documents. Similarly, where overlap occurs with other guidelines for valvular interventions, reference is made to parallel publications. There is a paucity of high-level quality of evidence in the form of randomized clinical trials to support guidelines in ACHD. We accounted for this in the wording of the strength of recommendations put forth by our national and international experts. As data grow on long-term follow-up, we expect that the evidence driving clinical practice will become increasingly granular. These recommendations are meant to be used to guide dialogue between clinicians, interventional cardiologists, surgeons, and patients making complex decisions relative to ACHD interventions.

Background: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy. However, the efficacy and safety of this invasive therapy in the prevention of recurrent ICD shocks in patients with TOF has not been well evaluated. Methods: Records of a total of 47 consecutive TOF patients (mean age: 43.1 ± 13.2 years, male sex: n = 34 [72.3%]) who underwent ICD implantation for secondary prevention between 2000 and 2018 were reviewed. Results: Twenty (42.6%) patients underwent invasive therapy (radiofrequency catheter ablation, n = 8; surgical ablation with pulmonary valve replacement, n = 12) before ICD implantation. Twenty-seven patients (57.4%) were managed noninvasively. During follow-up (median 80.5 [interquartile range, 28.5-131.0] months), 2 (10.0%) patients in the invasive group and 10 (37.0%) patients in the noninvasive group received appropriate ICD shocks (P = 0.036). Logistic regression analysis showed that invasive therapy was associated with a decreased risk of ICD shocks by 81.1% (odds ratio, 0.189; 95% confidence interval, 0.036-0.990; P = 0.049). Furthermore, invasive therapy was associated with decreased risk of the composite outcomes of ICD shock, death, cardiac transplantation, and hospital admission (odds ratio, 0.090; 95% confidence interval, 0.025-0.365; P = 0.013) compared with noninvasive therapy. Conclusions: Invasive substrate modification therapy was associated with a lower likelihood of ICD shocks and improvement of long-term outcomes in TOF patients. Résumé: Contexte: Les défibrillateurs cardioverteurs implantables (DCI) sont efficaces pour prévenir la mort cardiaque subite provoquée par une arythmie chez les patients présentant une tétralogie de Fallot (TF). Bien que le traitement des arythmies ventriculaires malignes par DCI puisse sauver des vies, les chocs administrés peuvent avoir des conséquences délétères. L’ablation du substrat est devenue le traitement de référence pour prévenir l’administration à répétition de chocs par DCI chez les patients atteints d’une cardiomyopathie ischémique. L’efficacité et l’innocuité de ce traitement invasif pour prévenir l’administration de chocs répétés chez les patients présentant une TF n’ont toutefois pas été bien évaluées. Méthodologie: Nous avons examiné les cas consécutifs de 47 patients présentant une TF (âge moyen : 43,1 ± 13,2 ans; hommes : n = 34 [72,3 %]) ayant reçu un DCI en prévention secondaire entre 2000 et 2018. Résultats: Au total, 20 (42,6 %) patients ont subi un traitement invasif (ablation par cathéter par radiofréquence, n = 8; ablation chirurgicale et remplacement de la valve pulmonaire, n = 12) avant l’implantation d’un DCI. Vingt-sept patients (57,4 %) ont été pris en charge de façon non invasive. Au cours de la période de suivi (durée médiane de 80,5 [intervalle interquartile : 28,5 à 131,0] mois), 2 (10,0 %) patients du groupe ayant subi une intervention invasive et 10 (37,0 %) patients du groupe ayant subi une intervention non invasive ont reçu un choc approprié par DCI (p = 0,036). Les résultats de l’analyse par régression logistique montrent que le traitement invasif est associé à une réduction du risque de choc par DCI de 81,1 % (rapport des cotes : 0,189; intervalle de confiance à 95 % : de 0,036 à 0,990; p = 0,049). En outre, le traitement invasif est associé à une réduction du risque de survenue d’un des événements du paramètre d’évaluation composé, soit un choc administré par DCI, le décès, une transplantation cardiaque ou une hospitalisation (rapport des cotes : 0,090; intervalle de confiance à 95 % : de 0,025 à 0,365; p = 0,013) par rapport au traitement non invasif. Conclusions: La modification invasive du substrat a été associée à une probabilité plus faible de choc administré par DCI et à une amélioration des résultats à long terme chez les patients présentant une TF.

Introduction: There are numerous challenges to catheter ablation in patients with congenital heart disease (CHD), including access to cardiac chambers, distorted anatomies, displaced conduction systems, multiple and/or complex arrhythmia substrates, and excessively thickened walls, or interposed material. Areas covered: Herein, we review recent developments in catheter ablation strategies for patients with CHD that are helpful in addressing these challenges. Expert opinion: Remote magnetic navigation overcomes many challenges associated with vascular obstructions, chamber access, and catheter contact. Patients with CHD may benefit from a range of ablation catheter technologies, including irrigated-tip and contact-force radiofrequency ablation and focal and balloon cryoablation. High-density mapping, along with advances in multipolar catheters and interpolation algorithms, is contributing to new mechanistic insights into complex arrhythmias. Ripple mapping allows the activation wave front to be tracked visually without prior assignment of local activation times or window of interest, and without interpolations of unmapped regions. There is growing interest in measuring conduction velocities to identify arrhythmogenic substrates. Noninvasive mapping with a multielectrode-embedded vest allows prolonged bedside monitoring, which is of particular interest in those with non-sustained or multiple arrhythmias. Further studies are required to assess the role of radiofrequency needle catheters and stereotactic radiotherapy in patients with CHD.

BackgroundPatients with Fontan circulation are known to be at high risk for developing atrial tachyarrhythmias (AAs). Our objective was to examine the efficacy and safety of amiodarone in the management of ATs in adult Fontan patients.MethodsPrimary outcomes of this single-centre, retrospective study included freedom from AAs and incidence of adverse effects of amiodarone on Fontan patients. Heart failure (HF) events and composite outcomes of death from any cause, Fontan revision and heart transplantation were evaluated as secondary outcomes. Predictors of HF and discontinuing amiodarone were also evaluated.ResultsA total of 61 patients (mean age 31.6±11.3 years, 40.9% female), who were treated with amiodarone in between 1995 and 2018, were included. AAs free survival at 1, 3 and 5 years were 76.2%, 56.9% and 30.6%, respectively. During a median follow-up of 50.5 months, 34 (55.7%) patients developed side effects, and 20 (32.8%) patients discontinued amiodarone due to side effects. Thyroid dysfunction was the most common side effect (n=26, 76.5%), amiodarone-induced thyrotoxicosis (AIT) (n=16, 27.1%) being most common thyroid dysfunction. Young age (age ConclusionsShort-term efficacy of amiodarone in Fontan physiology is acceptable. However, long-term administration is associated with a reduction of efficacy and a significant prevalence of non-cardiac side effects. AIT is associated with exacerbation of HF. The judicious use of amiodarone administration should be considered in this population.

Background Left ventricular (LV) lead implantation for cardiac resynchronization therapy (CRT) may be confounded by contrast load during attempted cannulation and lead dislodgement during guiding catheter splitting. An LV lead implant system with a steerable single catheter that completely avoids the use of guiding catheters when needed, acquires atrioventricular electrograms, measures intracardiac pressures, permits CS angiography, and has the ability to direct a coronary angioplasty wire that will lead the final delivery of LV lead into a CS tributary, may help limit contrast use and avoid lead dislodgement at CS guide sheath removal. Methods and results In this article as a proof of concept, we describe the use of this minimalist technique as a first line approach in six patients who had standard indications for CRT. The LV lead was successfully implanted in a target vein in all patients without acute complications. Contrast was not used in half the group and the LV lead was successfully implanted without guiding catheter in four patients. The implantation technique evolved through the series and in the final patient, no guiding sheath or contrast was used. Postimplant lead positions on chest X-ray and lead parameters were stable in all patients at follow-up. Conclusion In proof of concept paper, we describe a technique of LV lead implantation potentially without the use of contrast and standard CS guiding catheters. Once familiar, this approach may provide a less complicated strategy.

Atrial arrhythmias are highly prevalent in the aging Fontan population and contribute importantly to morbidity and mortality. Although the most common arrhythmia is scar-based intra-atrial re-entrant tachycardia, various other arrhythmias may occur, including focal atrial tachycardia, atrioventricular node-dependent tachycardias, and atrial fibrillation. The type and prevalence of atrial arrhythmia is determined, in part, by the underlying congenital defect and variant of Fontan surgery. Although the cumulative incidence of atrial tachyarrhythmias has decreased substantially from the atriopulmonary anastomosis to the more recent total cavopulmonary-connection Fontan, the burden of atrial arrhythmias remains substantial. Management is often multifaceted and can include anticoagulation, anti-arrhythmic drug therapy, pacing, and cardioversion. Catheter ablation plays a key role in control of arrhythmia. Risks and benefits must be carefully weighed. Among the important considerations are the clinical burden of arrhythmia, ventricular function, hemodynamic stability in tachycardia, suspected arrhythmia mechanisms, risks associated with anaesthesia, venous access, approaches to reaching the pulmonary venous atrium, and accompanying comorbidities. Careful review of surgical notes, electrocardiographic tracings, and advanced imaging is paramount, with particular attention to anatomic abnormalities such as venous obstructions and displaced conduction systems. Despite numerous challenges, ablation of atrial arrhythmias is effective in improving clinical status. Nevertheless, onset of new arrhythmias is common during long-term follow-up. Advanced technologies, such as high-density mapping catheters and remote magnetic guided ablation, carry the potential to further improve outcomes. Fontan patients with atrial arrhythmias should be referred to centres with dedicated expertise in congenital heart disease including catheter ablation, anaesthesia support, and advanced imaging.

A 33‐year‐old male who underwent surgery for Tetralogy of Fallot presented with atrial flutter. Electrophysiology study revealed concealed entrainment along the mid lateral right atrium with postpacing interval shorter than the tachycardia cycle length. Ablation at this site terminated the tachycardia. The presence of shorter PPI than TCL was due to a large virtual electrode leading to downstream capture of far field tissue. This case demonstrates that sites showing PPI shorter than TCL are in a slow conducting narrow critical isthmus and hence constitute good ablation targets.