Arrhythmias in adult patients after Rastelli surgery: a single-centre experience.

BACKGROUND: Rastelli surgery is used for the correction of several CHDs. Although late-onset cardiac arrhythmias have emerged as a major complication after corrective surgeries, there is a paucity of data on arrhythmias after Rastelli surgery. METHODS: This retrospective cohort study was conducted on patients who had undergone Rastelli surgery and have been followed at the adult CHD clinic at our hospital. RESULTS: A total of 55 patients (36.4% female, age 22.2 ± 6.4 years) were followed for a median period of 24.2 (20.6-31.0) years. Tachyarrhythmias occurred in 21 (38.4 %) patients (n = 15 for atrial tachycardia, 5 for ventricular tachycardia, and 1 for both atrial and ventricular tachycardia). Older age at surgery was significantly associated with the risk of tachyarrhythmias (P = 0.022). Bradyarrhythmia occurred in 12 (21.8%) patients and consisted of perioperative AV block (n = 5), late AV block (n = 1), and sinus node dysfunction (n = 6). Nine (16.4%) patients underwent catheter ablation. The mechanisms of atrial arrhythmias include cavotricuspid isthmus-dependent and surgical scar-dependent intra-atrial reentrant tachycardias. Among the three patients who underwent ablation for ventricular tachycardia, all circuits were dependent on the scar at the base of the right ventricle to pulmonary artery conduit. Median survival free from any event (arrhythmia, death, or heart failure) was 31.6 (28.1-35.1) years after Rastelli surgery. CONCLUSIONS: The prevalence of arrhythmias late after Rastelli surgery is substantial and increases in the second decade after surgery. Older age at surgery is associated with a higher prevalence of arrhythmias.

Minimalistic strategy for coronary sinus lead implant: A single tool capable of electrophysiological mapping, pressure measurement, and angiography.

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.

The effect of left ventricular pacing on transmural activation delay in myopathic human hearts.

Aims: Left ventricular (LV) epicardial pacing (LVEpiP) in human myopathic hearts does not decrease global epicardial activation delay compared with right ventricular (RV) endocardial pacing (RVEndoP); however, the effect on transmural activation delay has not been evaluated. To characterize the transmural electrical activation delay in human myopathic hearts during RVEndoP and LVEpiP compared with global epicardial activation delay. Methods and results: Explanted hearts from seven patients (5 male, 46 ± 10 years) undergoing cardiac transplantation were Langendorff-perfused and mapped using an epicardial sock electrode array (112 electrodes) and 25 transmural plunge needles (four electrodes, 2 mm spacing), for a total of 100 unipolar transmural electrodes. Electrograms were recorded during LVEpiP and RVEndoP, and epicardial (sock) and transmural (needle) activation times, along with patterns of activation, were compared. There was no difference between the global epicardial activation times (LVEpiP 147 ± 8 ms vs. RVEndoP 156 ± 17 ms, P = 0.46). The mean LV transmural activation time during LVEpiP was significantly shorter than that during RVEndoP (125 ± 44 vs. 172 ± 43 ms, P < 0.001). During LVEpiP, of the transmural layers endo-, mid-myocardium and epicardium, LV endocardial layer was often the earliest compared with other transmural layers. Conclusion: In myopathic human hearts, LVEpiP did not decrease global epicardial activation delays compared with RVEndoP. LV epicardial pacing led to early activation of the LV endocardium, revealing the importance of the LV endocardium even when pacing from the LV epicardium.

When Ventricular Safety Standby Does Not Work.

Dual chamber pacemakers have inbuilt advanced safety systems such as ventricular safety standby (crosstalk detection) to prevent ventricular oversensing resulting in inappropriate pacing inhibition. We describe a case where this safety mechanism does not reliably work and the management required to rectify the situation in an educational format.

Bipolar ablation for deep intra-myocardial circuits: human ex vivo development and in vivo experience.

AIMS: Current conventional ablation strategies for ventricular tachycardia (VT) aim to interrupt reentrant circuits by creating ablation lesions. However, the critical components of reentrant VT circuits may be located at deep intramural sites. We hypothesized that bipolar ablations would create deeper lesions than unipolar ablation in human hearts. METHODS AND RESULTS: Ablation was performed on nine explanted human hearts at the time of transplantation. Following explant, the hearts were perfused by using a Langendorff perfusion setup. For bipolar ablation, the endocardial catheter was connected to the generator as the active electrode and the epicardial catheter as the return electrode. Unipolar ablation was performed at 50 W with irrigation of 25 mL/min, with temperature limit of 50°C. Bipolar ablation was performed with the same settings. Subsequently, in a patient with an incessant septal VT, catheters were positioned on the septum from both the ventricles and radiofrequency was delivered with 40 W. In the explanted hearts, there were a total of nine unipolar ablations and four bipolar ablations. The lesion depth was greater with bipolar ablation, 14.8 vs. 6.1 mm (P < 0.01), but the width was not different (9.8 vs. 7.8 mm). All bipolar lesions achieved transmurality in contrast to the unipolar ablations. In the patient with a septal focus, bipolar ablation resulted in termination of VT with no inducible VTs. CONCLUSION: By using a bipolar ablation technique, we have demonstrated the creation of significantly deeper lesions without increasing the lesion width, compared with standard ablation. Further clinical trials are warranted to detail the risks of this technique.

Pacing inhibition from inter-coronary wire interaction during angioplasty.

Electro-mechanical noise from the interaction of multiple coronary wires can inhibit pacing with a risk of asystole in pacemaker-dependent patients. Awareness of this phenomenon is important to guide prompt management with adjustment of device sensitivity, asynchronous pacing, removal of a coronary wire or insertion of a temporary pacing system.

Role of subpulmonary right ventricle in sudden cardiac death in adults with congenital heart disease.

Despite improved childhood survival of congenital heart disease (CHD) as a result of advances in management, late-onset sudden cardiac death (SCD) from malignant ventricular arrhythmias remains a leading cause of mortality in adults with CHD. Preventing SCD in these patients requires an understanding of the underlying pathophysiological mechanisms. Many CHD patients experience significant hemodynamic stress on the subpulmonary right ventricle (RV), leading to pathologic remodeling. Unlike acquired heart disease in which left ventricular pathology is prevalent, RV pathologies are crucial in the SCD pathogenesis in CHD patients. This review examines the mechanisms and management of SCD related to subpulmonary RV pathologies in CHD patients.

Incremental value of machine learning for risk prediction in tetralogy of Fallot.

OBJECTIVE: Machine learning (ML) can facilitate prediction of major adverse cardiovascular events (MACEs) in repaired tetralogy of Fallot (rTOF). We sought to determine the incremental value of ML above expert clinical judgement for risk prediction in rTOF. METHODS: Adult congenital heart disease (ACHD) clinicians (≥10 years of experience) participated (one cardiac surgeon and four cardiologists (two paediatric and two adult cardiology trained) with expertise in heart failure (HF), electrophysiology, imaging and intervention). Clinicians identified 10 high-yield variables for 5-year MACE prediction (defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia and HF). Risk for MACE (low, moderate or high) was assigned by clinicians blinded to outcome for adults with rTOF identified from an institutional database (n=25 patient reviews conducted by five independent observers). A validated ML model identified 10 variables for risk prediction in the same population. RESULTS: Prediction by ML was similar to the aggregate score of all experts (area under the curve (AUC) 0.85 (95% CI 0.58 to 0.96) vs 0.92 (0.72 to 0.98), p=0.315). Experts with ≥20 years of experience had superior discriminative capacity compared with <20 years (AUC 0.98 (95% CI 0.86 to 0.99) vs 0.80 (0.56 to 0.93), p=0.027). In those with <20 years of experience, ML provided incremental value such that the combined (clinical+ML) AUC approached ≥20 years (AUC 0.85 (95% CI 0.61 to 0.95), p=0.055). CONCLUSIONS: Robust prediction of 5-year MACE in rTOF was achieved using either ML or a multidisciplinary team of ACHD experts. Risk prediction of some clinicians was enhanced by incorporation of ML suggesting that there may be incremental value for ML in select circumstances.

Role of KATP channels in the maintenance of ventricular fibrillation in cardiomyopathic human hearts.

RATIONALE: Ventricular fibrillation (VF) leads to global ischemia. The modulation of ischemia-dependent pathways may alter the electrophysiological evolution of VF. OBJECTIVE: We addressed the hypotheses that there is regional disease-related expression of K(ATP) channels in human cardiomyopathic hearts and that K(ATP) channel blockade promotes spontaneous VF termination by attenuating spatiotemporal dispersion of refractoriness. METHODS AND RESULTS: In a human Langendorff model, electric mapping of 6 control and 9 treatment (10 µmol/L glibenclamide) isolated cardiomyopathic hearts was performed. Spontaneous defibrillation was studied and mean VF cycle length was compared regionally at VF onset and after 180 seconds between control and treatment groups. K(ATP) subunit gene expression was compared between LV endocardium versus epicardium in myopathic hearts. Spontaneous VF termination occurred in 1 of 6 control hearts and 7 of 8 glibenclamide-treated hearts (P=0.026). After 180 seconds of ischemia, a transmural dispersion in VF cycle length was observed between epicardium and endocardium (P=0.001), which was attenuated by glibenclamide. There was greater gene expression of all K(ATP) subunit on the endocardium compared with the epicardium (P<0.02). In an ischemic rat heart model, transmural dispersion of refractoriness (ΔERP(Transmural)=ERP(Epicardium)-ERP(Endocardium)) was verified with pacing protocols. ΔERP(Transmural) in control was 5 ± 2 ms and increased to 36 ± 5 ms with ischemia. This effect was greatly attenuated by glibenclamide (ΔERP(Transmural) for glibenclamide+ischemia=4.9 ± 4 ms, P=0.019 versus control ischemia). CONCLUSIONS: K(ATP) channel subunit gene expression is heterogeneously altered in the cardiomyopathic human heart. Blockade of K(ATP) channels promotes spontaneous defibrillation in cardiomyopathic human hearts by attenuating the ischemia-dependent spatiotemporal heterogeneity of refractoriness during early VF.

Incidence and Clinical Predictors of Early and Late Complications of Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease.

BACKGROUND: The implantable cardioverter-defibrillator (ICD) has been proven to improve survival in adults with congenital heart disease (ACHD), but it is associated with a high rate of complications. We aimed to quantify the incidence of early (≤ 3 months; ECs) and late (> 3 months; LCs) complications in ACHD patients implanted with an ICD and to identify their clinical predictors. METHODS: We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General Hospital from 1996 to 2019. RESULTS: The most common diagnoses were tetralogy of Fallot (32.4%), dextro-transposition of the great arteries (17.9%), and congenital corrected transposition of the great arteries (13%). No intraprocedural complications were observed. Median follow-up was 3.4 years (IQR 0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgements). A total of 56 LCs occurred (46% lead failure, 21% infection, 11% prophylactic lead extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LCs of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9; P = 0.02) and cardiac resynchronization therapy defibrillator implant (odds ratio 2.5; P = 0.04) were associated with ECs. Survival rates free from LCs were 92%, 86%, and 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, hazard ratio 1.35; P = 0.031) were associated with LCs. CONCLUSIONS: ACHD patients at risk of sudden cardiac death 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.

Risk Stratification for Sudden Cardiac Death in Repaired Tetralogy of Fallot.

There has been significant progress in the prevention of sudden cardiac death in repaired tetralogy of Fallot. Contemporary cohorts report greater survival attributable to improved surgical techniques, heart failure management, and proactive strategies for risk stratification and management of ventricular arrhythmias including defibrillator implantation and ablation technology. Over the last 25 years, our understanding of predictive risk factors has also improved from invasive and more limited measures to individualized risk prediction scores based on extensive demographic, imaging, electrophysiological, and functional data. Although each of these contemporary scoring systems improves prediction, there are important differences between the study cohorts, included risk factors, and imaging modalities that can significantly affect interpretation and implementation for the individual patient. In addition, accurate phenotyping of disease complexity and anatomic repair substantially modulates this risk and the mechanism of sudden death. Routine implementation of risk stratification within repaired tetralogy of Fallot management is important and directly informs primary prevention defibrillator implantation as well as consideration for proactive invasive strategies including ventricular tachycardia ablation and pulmonary valve replacement. Assessment and risk stratification by a multidisciplinary team of experts in adult congenital heart disease are crucial and critical. Although we have increased understanding, reconciliation of these complex factors for the individual patient remains challenging and often requires careful consideration and discussion with multidisciplinary teams, patients, and their families.

De grands progrès ont été réalisés pour prévenir la mort subite d'origine cardiaque chez les patients ayant une tétralogie de Fallot réparée (TFr). Dans les cohortes contemporaines, l'amélioration du taux de survie peut être attribuée à l'évolution des techniques chirurgicales, à la prise en charge de l'insuffisance cardiaque et à la mise en place de stratégies proactives pour la stratification du risque d'arythmies ventriculaires et pour leur prise en charge, notamment par l'implantation de défibrillateurs et l'ablation. Au cours de 25 dernières années, les moyens utilisés pour caractériser les facteurs de risque à valeur prédictive sont passés de mesures limitées et invasives à l'établissement de scores individualisés basés sur de grands corpus de données démographiques, électrophysiologiques, fonctionnelles et d'autres issues de l'imagerie. Bien que chacun de ces systèmes contemporains d'évaluation du risque permette de raffiner notre capacité prédictive, des différences importantes entre les cohortes à l'étude, les facteurs de risque considérés et les modalités d'imagerie peuvent influencer l'interprétation des scores et les soins prodigués à un patient en particulier. De plus, la description phénotypique exacte de la complexité de la maladie et de la réparation anatomique permet de moduler la stratification du risque de mort subite d'origine cardiaque et son mécanisme possible. Il importe que la stratification du risque fasse partie intégrante de la prise en charge de la TFr puisqu'elle oriente directement le choix de mettre ou non en place un défibrillateur en prévention primaire, et qu'elle fasse partie de l'équation lorsque des stratégies invasives proactives, comme l'ablation de la tachycardie ventriculaire ou le remplacement de la valve pulmonaire, sont envisagées. La mesure et la stratification du risque par une équipe multidisciplinaire d'experts en cardiopathies congénitales sont donc des étapes cruciales. Même si les connaissances se sont affinées au fil du temps, il peut être difficile de faire la synthèse de ces facteurs complexes dans le cas d'un patient en particulier. C'est pourquoi il faut bien souvent se tourner vers l'équipe multidisciplinaire, le patient et ses proches pour évaluer rigoureusement les options.

Ebstein's Anomaly: How to Identify Intracardiac Structures When Electrical Signals Do Not Match the Anatomy.

In patients with Ebstein's anomaly, the distorted anatomy with discordance between the true atrioventricular (AV) groove and the tricuspid valve poses many challenges to the electrophysiologist. Intracardiac echocardiography is a recent tool that allows visualization of the displaced tricuspid valve, the true AV groove, and the atrialized right ventricle. We present a 3-dimensional electroanatomic map built using intracardiac echocardiography and the CARTOSOUND® module (Biosense Webster, Diamond Bar, CA, USA) in one such patient who underwent ablation of a right-sided mid-septal accessory pathway.

Catheter Ablation of Atrial Arrhythmias in Patients Post-Fontan.

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.

Quantifying the impact of equity, diversity, and inclusion in electrophysiology: Training the first female electrophysiologists from Jamaica and Saint Lucia.

Background: 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. Objective: 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. Methods: 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. Results: 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. Conclusion: 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.

Automated Identification of Ventricular Tachycardia Ablation Targets: Multicenter Validation and Workflow Characterization.

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.

Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults With Congenital Heart Disease.

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.