Congenital Electrophysiology Research Articles

Assessment of current pediatric electrocardiogram (ECG) interpretation practices

BackgroundDespite improvements in digital electrocardiograms (ECGs), current standard of care requires physician confirmation. Mismatched expectations between ordering providers and ECG readers, often pediatric cardiologists and electrophysiologists (EPs), are common, especially since there are no standardized practices for pediatric ECG reading. ObjectivesThe aim of this study was to understand current practices in pediatric ECG reading. MethodsAn electronic survey was sent to members of the Pediatric and Congenital Electrophysiology Society (PACES). Participation was optional; results were recorded from 12/19/22–1/9/23. Only complete and non-duplicate entries were included. ResultsA total of 127 responses were received, 93 were analyzed. Most responses were from centers in North America (n = 65, 70 %), including the US (n = 58, 62 %), Canada (n = 6, 6 %), and Mexico (n = 1, 1 %). The remaining were from Europe (n = 18, 19 %), Asia (n = 7, 8 %), Australia (n = 2, 2 %), and South America (n = 1, 1 %). Most (n = 46, 49 %) were from small centers (0–25 ECGs read per day), 27 respondents (29 %) were from medium centers (26–50 ECGs read per day), 20 respondents (22 %) were from large centers (>50 ECGs read per day). The majority (n = 65, 70 %) reported >3 readers/day for inpatient and emergency department ECGs. 49 % (n = 46) of centers read ECGs >2 times/day on weekdays with more variable practice on weekends. For critical/time sensitive findings, most centers (n = 90, 97 %) used verbal communication +/− the EMR. There was consensus (≥50 % agreement) that the following findings are critical/time sensitive: QTc >500 ms, T-wave alternans, narrow complex tachycardia, wide complex tachycardia, pre-excited atrial fibrillation, focal ischemic changes, second degree heart block type II, complete heart block, and pacemaker malfunction. ConclusionReading practices are variable. Critical/time sensitive findings are most often communicated verbally, however, there is no agreed upon standard. There was consensus in critical/time-sensitive findings. Improved understanding of common practices and resource allocation may lead to increased consistency in pediatric ECG reading.

Remote magnetic navigation for catheter ablation of complex arrhythmias in congenital heart disease: an initial experience using a transnational telerobotic approach

Abstract Background The field of congenital electrophysiology (EP) is still in its relative infacncy with a limited number of electrophysiologists capable of performing catheter ablation in patients with congenital heart disease (CHD). As a result, patients or physicians are often required to travel great distances to performed specialised ablation procedures, something made near impossible during the COVID-19 pandemic. Objective We aimed to explore the feasibility and safety of a telerobotic approach to catheter ablation where the remote operator was situated approximately 1200km away from the patient and local team. Methods A 3D-printed 'phantom' heart was used in a pilot experiment in which no significant difference was seen between the local and remote operators in electroanatomic (EAM) mapping of the phantom heart using remote magnetic navigation (RMN). Catheter ablation was subsequently performed in patients with severe CHD using a transnational telerobotic approach to catheter ablation where the remote operator was able to view the dedicated console with live fluoroscopy, EAM and EP signals, as well as control the mapping and ablation catheters from 1200km away. As a safety precaution, radiofrequency (RF) could only be delivered by the local team. Results In total, 10 procedures were performed in 9 patients with severe CHD (mean age 38) between April and December 2021. All patients underwent an atrial ablation and one additional non-CHD patient underwent denervation of the pulmonary arteries. Median procedure duration was 335 (minutes) (IWR 230-349) with 11.5 minutes (QRS 3-15.4) of fluoroscopy exposure and 752 (IQR 674-905) seconds of RF delivery. There were no acute procedural complications. At one-year follow-up, one patient died as a result of heart failure and there were two arrhythmia recurrences. Conclusions Telerobotic catheter ablation is feasible and effective and creates the possibility of exciting new approaches to remote ablation and its use in the tele-proctoring of international fellows.

Long-Term Outcomes of Cardiac Resynchronization Therapy in Patients With Repaired Tetralogy of Fallot: A Multicenter Study.

A growing number of patients with tetralogy of Fallot develop left ventricular systolic dysfunction and heart failure, in addition to right ventricular dysfunction. Although cardiac resynchronization therapy (CRT) is an established treatment option, the effect of CRT in this population is still not well defined. This study aimed to investigate the early and late efficacy, survival, and safety of CRT in patients with tetralogy of Fallot. Data were analyzed from an observational, retrospective, multicenter cohort, initiated jointly by the Pediatric and Congenital Electrophysiology Society and the International Society of Adult Congenital Heart Disease. Twelve centers contributed baseline and longitudinal data, including vital status, left ventricular ejection fraction (LVEF), QRS duration, and NYHA functional class. Outcomes were analyzed at early (3 months), intermediate (1 year), and late follow-up (≥2 years) after CRT implantation. A total of 44 patients (40.3±19.2 years) with tetralogy of Fallot and CRT were enrolled. Twenty-nine (65.9%) patients had right ventricular pacing before CRT upgrade. The left ventricular ejection fraction improved from 32% [24%-44%] at baseline to 42% [32%-50%] at early follow-up (P<0.001) and remained improved from baseline thereafter (P≤0.002). The QRS duration decreased from 180 [160-205] ms at baseline to 152 [133-182] ms at early follow-up (P<0.001) and remained decreased at intermediate and late follow-up (P≤0.001). Patients with upgraded CRT had consistent improvement in left ventricular ejection fraction and QRS duration at each time point (P≤0.004). Patients had a significantly improved New York Heart Association functional class after CRT implantation at each time point compared with baseline (P≤0.002). The transplant-free survival rates at 3, 5, and 8 years after CRT implantation were 85%, 79%, and 73%. In patients with tetralogy of Fallot treated with CRT consistent improvement in QRS duration, left ventricular ejection fraction, New York Heart Association functional class, and reasonable long-term survival were observed. The findings from this multicenter study support the consideration of CRT in this unique population.

Pharmacological Management of Cardiac Arrhythmias in the Fetal and Neonatal Periods: A Scientific Statement From the American Heart Association: Endorsed by the Pediatric & Congenital Electrophysiology Society (PACES).

Disorders of the cardiac rhythm may occur in both the fetus and neonate. Because of the immature myocardium, the hemodynamic consequences of either bradyarrhythmias or tachyarrhythmias may be far more significant than in mature physiological states. Treatment options are limited in the fetus and neonate because of limited vascular access, patient size, and the significant risk/benefit ratio of any intervention. In addition, exposure of the fetus or neonate to either persistent arrhythmias or antiarrhythmic medications may have yet-to-be-determined long-term developmental consequences. This scientific statement discusses the mechanism of arrhythmias, pharmacological treatment options, and distinct aspects of pharmacokinetics for the fetus and neonate. From the available current data, subjects of apparent consistency/consensus are presented, as well as future directions for research in terms of aspects of care for which evidence has not been established.

Thromboembolism prophylaxis practices of pediatric and congenital electrophysiologists during invasive electrophysiology studies: A PACES survey.

Thromboembolic events related to invasive electrophysiology studies, while rare, can have devastating consequences. Use of systemic anticoagulation for a pediatric or adult-congenital invasive electrophysiology study is recommended, however there is no established standard of practice in this population. To report on procedural practices for thromboembolism prophylaxis during invasive electrophysiology studies for pediatric patients and adults with congenital heart disease. An anonymous web-based survey was sent to the members of the Pediatric and Congenital Electrophysiology Society. The survey focused on pre-procedural, intra-procedural, and post-procedural thromboembolism prophylaxis practices during invasive electrophysiology studies. Significant practice variation was defined as <90% concordance among respondents. Survey was completed by 73 members; 52 (71%) practicing in the United States, 65 (89%) practicing in an academic institution, and 14 (19%) in an institution that performs more than 200 invasive electrophysiology procedures annually. Responses showed significant variation in practice. Prior to an invasive electrophysiology procedure, 25% discontinue aspirin while 47% discontinue anticoagulants. Heparin is given for all procedures by 32%. When heparin is administered, the first dose is given by 32% after sheaths are placed, 42% after crossing into the systemic atrium, and 26% just prior to systemic-side ablation. Most target an activated clotting time between 200-300 seconds. Post systemic-side ablation, 58% do not initiate a heparin infusion. Post-procedural oral agents were initiated on day of procedure by 34% of respondents and on post-procedure day 1 by 53%. If treating with aspirin, 74% use low-dose (3-5mg/kg or 81mg daily), and 68% treat for 4-6weeks. There is significant variation in thromboembolism prophylaxis for invasive EP studies among pediatric and congenital electrophysiologists. Further studies are needed to optimize the management of thromboembolism prophylaxis in this population.

Current State of Cardiac Implantable Electronic Device Remote Monitoring in Pediatrics and Congenital Heart Disease: A PACES-Sponsored Quality Improvement Initiative.

Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring(RM) data among pediatric and ACHD centers prior to implementation ofa Pediatric and Congenital Electrophysiology Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors(ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.

Editorial introductions.

Editorial introductions.

PO-03-147 EVALUATION OF A PEDIATRIC ELECTROPHYSIOLOGY CASE COMPLEXITY PREDICTION MODEL TO OPTIMIZE PROCEDURAL SCHEDULING

While operational efficiency has been evaluated in adult interventional and surgical specialties, limited data exist regarding the prediction of case complexity in the pediatric and congenital electrophysiology (EP) laboratory. To identify pre-procedural variables that predict prolonged case durations among pediatric and adult congenital patients presenting for diagnostic EP study and catheter ablation. A retrospective cross-sectional study was performed of all EP studies performed at Boston Children’s Hospital between 1/1/2015-1/1/2020 including pediatric and adult congenital heart disease patients of all ages. The primary outcome was case duration ≥180 minutes. Univariate and multivariate analyses were performed for the primary outcome using logistic regression, with a sub-analysis repeated for pediatric patients <20 years of age. During the 5-year period, 1025 patients underwent EP study and 292 (28.5%) had a case duration ≥180 minutes. Factors associated with the primary outcome on multivariate analysis were: age ≥20 years at EP study (OR 1.95, 95% CI 1.24-3.07, p 0.004), arrhythmia diagnosis of premature ventricular contractions/ventricular tachycardia (PVC/VT, OR 3.70, 95% CI 2.10-6.55, p <0.001), Fontan (OR 3.74, 95% CI 1.69-8.30, p=0.001) or Mustard/Senning physiology (OR 4.13, 95% CI 1.48-11.60, p=0.007), and the presence of non-cardiac comorbidities (OR 2.47, 95% CI 1.29-4.73, p 0.006). Restricting the analysis to pediatric patients <20 years old, factors associated with the primary outcome on multivariate analysis were an arrhythmia diagnosis of PVC/VT (OR 3.04, 95% CI 1.64-5.63, p <0.001), Fontan physiology (OR 3.13, 95% CI 1.10-8.92, p 0.033), non-cardiac comorbidities (OR 4.32, 95% CI 2.07-9.01, p <0.001), and prior ablation (OR 1.58, 95% CI 1.03-2.41, p 0.035). Intra-atrial reentrant tachycardia was not associated with prolonged case duration in either multivariate model. A weighted risk score using factors identified on multivariate analyses correctly classified procedures with prolonged case duration with reasonable accuracy (c-statistic 0.70). Age-dependent anatomic, electrophysiologic, and comorbid factors are associated with prolonged case duration in the pediatric and congenital EP laboratory. Applying a simple risk score using pre-procedural variables can discriminate cases with and without prolonged duration, and may aid in efficient procedural scheduling practices.

Transcatheter Leadless Pacing in Children: A PACES Collaborative Study in the Real-World Setting.

Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population. The objectives of this study are to evaluate clinical indications, procedural characteristics, electrical performance, and outcomes of TLP implantation in children. Retrospective data were collected from patients enrolled in the Pediatric and Congenital Electrophysiology Society TLP registry involving 15 centers. Patients ≤21 years of age who underwent Micra (Medtronic Inc, Minneapolis, MN) TLP implantation and had follow-up of ≥1 week were included in the study. The device was successfully implanted in 62 of 63 registry patients (98%) at a mean age of 15±4.1 years and included 20 (32%) patients with congenital heart disease. The mean body weight at TLP implantation was 55±19 kg and included 8 patients ≤8 years of age and ≤30 kg in weight. TLP was implanted by femoral (n=55, 87%) and internal jugular (n=8, 12.6%) venous approaches. During a mean follow-up period of 9.5±5.3 months, there were 10 (16%) complications including one cardiac perforation/pericardial effusion, one nonocclusive femoral venous thrombus, and one retrieval and replacement of TLP due to high thresholds. There were no deaths, TLP infections, or device embolizations. Electrical parameters, including capture thresholds, R wave sensing, and pacing impedances, remained stable. Initial results from the Pediatric and Congenital Electrophysiology Society TLP registry demonstrated a high level of successful Micra device implants via femoral and internal venous jugular approaches with stable electrical parameters and infrequent major complications. Long-term prospective data are needed to confirm the reproducibility of these initial findings.

Pediatric Electrophysiology Device Needs: A Survey from the Pediatric and Congenital Electrophysiology Society Taskforce on Pediatric-Specific Devices.

Background There are few US Food and Drug Administration (FDA)-approved devices specifically aimed at the pediatric patient with arrhythmia. This has led to a high off-label utilization of devices in this vulnerable population. The Pediatric and Congenital Electrophysiology Society (PACES), the international organization representing pediatric and congenital heart disease arrhythmia specialists, developed a task force to comprehensively address device development issues relevant to pediatric patients with congenital arrhythmia. Methods and Results As a first step, the taskforce developed a 26-question survey for the pediatric arrhythmia community to assess providers' understanding of the FDA approval process, specifically in regard to pediatric labeling. There were 92/211 respondents (44%) with a >90% completion rate. The vast majority of respondents believed there was a paucity of devices available for children (96%). More than 60% of respondents stated that they did not understand the FDA regulatory process and were not aware of whether the devices they used were labeled for pediatric use. Conclusions Pediatric electrophysiologists are keenly aware of the deficit of available pediatric devices for their patients. The majority do not understand the FDA approval process and could benefit from additional educational resources regarding this. A collaborative forum including PACES, FDA, patients and their families, and Industry would be an important next step in clarifying opportunities and priorities to serve this vulnerable population.

EP News: Pediatric and Congenital Electrophysiology.

Jorda et al (Eur Heart J 2022;43:3041, PMID 35766180) validated the ARVCrisk.com prediction model in an observational, retrospective, longitudinal study in an external multicenter cohort and compared its performance to current guideline recommendations for implantable cardioverter-defibrillator (ICD) use on the basis of decision curve analysis. (The ARVCrisk.com calculator is an online algorithm published in 2019 that intends to predict the occurrence of sustained ventricular arrhythmias [including sudden cardiac death or arrest] within 5 years of arrhythmogenic right ventricular cardiomyopathy diagnosis in patients aged 14 years.) The validation cohort included 429 patients (mean age 43 years), of whom 103 (24%) experienced sustained ventricular tachycardia during a median follow-up of 5 years.

EP News: Pediatric and Congenital Electrophysiology

Kannankeril et al (Pediatrics 2022;149:e2021054432, PMID 35284934) investigated the incidence of familial evaluation after sudden death, which is recommended by the Heart Rhythm Society for first-degree relatives after unexplained noninfant sudden death. Family members of decedents in the Sudden Death in the Young (SDY) Case Registry were queried to determine whether familial medical evaluation had occurred; cascade genetic testing was also offered. Of the 82 participants (related to 44 SDY cases), only 26% had plans to undergo medical evaluation for familial sudden death. DNA samples were provided by 76% of participants. No new diagnoses of inherited causes of sudden death were identified with family screening. The authors conclude that familial evaluation after SDY is inconsistent and the population-based method used for the study was not effective in identifying inherited heart disease. The education of families, primary care providers, and medical examiners may improve the use and utility of screening after SDY.

Catheter Ablation for Atrial Fibrillation in Adult Congenital Heart Disease: An International Multicenter Registry Study.

Data on atrial fibrillation (AF) ablation and outcomes are limited in patients with congenital heart disease (CHD). We aimed to investigate the characteristics of patients with CHD presenting for AF ablation and their outcomes. A multicenter, retrospective analysis was performed of patients with CHD undergoing AF ablation between 2004 and 2020 at 13 participating centers. The severity of CHD was classified using 2014 Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society guidelines. Clinical data were collected. One-year complete procedural success was defined as freedom from atrial tachycardia or AF in the absence of antiarrhythmic drugs or including previously failed antiarrhythmic drugs (partial success). Of 240 patients, 127 (53.4%) had persistent AF, 62.5% were male, and mean age was 55.2±13.3 years. CHD complexity categories included 147 (61.3%) simple, 68 (28.3%) intermediate, and 25 (10.4%) severe. The most common CHD type was atrial septal defect (n=78). More complex CHD conditions included transposition of the great arteries (n=14), anomalous pulmonary veins (n=13), tetralogy of Fallot (n=8), cor triatriatum (n=7), single ventricle physiology (n=2), among others. The majority (71.3%) of patients had trialed at least one antiarrhythmic drug. Forty-six patients (22.1%) had reduced systemic ventricular ejection fraction <50%, and mean left atrial diameter was 44.1±8.2 mm. Pulmonary vein isolation was performed in 227 patients (94.6%); additional ablation included left atrial linear ablations (40%), complex fractionated atrial electrogram (19.2%), and cavotricuspid isthmus ablation (40.8%). One-year complete and partial success rates were 45.0% and 20.5%, respectively, with no significant difference in the rate of complete success between complexity groups. Overall, 38 patients (15.8%) required more than one ablation procedure. There were 3 (1.3%) major and 13 (5.4%) minor procedural complications. AF ablation in CHD was safe and resulted in AF control in a majority of patients, regardless of complexity. Future work should address the most appropriate ablation targets in this challenging population.

EP News: Quality Improvement and Outcomes: Remote monitoring of pediatric cardiac implantable electronic devices

Remote monitoring of pediatric cardiac implantable electronic devices (CIEDs) has become an important tool in outpatient management. Recent guidelines (Shah et al, JACC Clin Electrophysiol 2021;7:1437, PMID 34794667) from the Pediatric and Congenital Electrophysiology Society (PACES) have recommended remote monitoring of CIEDs every 3–12 months for pacemakers and every 3–6 months for implantable cardioverter-defibrillators, with an increase in frequency when the device is approaching elective replacement indicators.

Rationale and Design of the Robotic Transcatheter Ablation in Congenital Heart Disease (ROBOTIC) Registry : from the Pediatric and Congenital Electrophysiology Society (PACES) and the International Society for Adult Congenital Heart Disease (ISACHD)

Rationale and Design of the Robotic Transcatheter Ablation in Congenital Heart Disease (ROBOTIC) Registry : from the Pediatric and Congenital Electrophysiology Society (PACES) and the International Society for Adult Congenital Heart Disease (ISACHD)

Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation

BackgroundPalliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPMv) may be associated with additional adverse long-term outcomes. ObjectivesThe goal of this study was to quantify the attributable risk of PPMv in patients with SV, and to identify modifiable risk factors. MethodsThis international study was sponsored by the Pediatric and Congenital Electrophysiology Society. Centers contributed baseline and longitudinal data for functionally SV patients with PPMv. Enrollment was at implantation. Controls were matched 1:1 to PPMv subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death. ResultsIn total, 236 PPMv subjects and 213 matched controls were identified (22 centers, 9 countries). Median age at enrollment was 5.3 years (quartiles: 1.5-13.2 years), follow-up 6.9 years (3.4-11.6 years). Median percent ventricular pacing (Vp) was 90.8% (25th-75th percentile: 4.3%-100%) in the PPMv cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPMv was 3.8 (95% CI 1.9-7.6; P < 0.001). Within the PPMv population, higher Vp (HR: 1.009 per %; P = 0.009), higher QRS z-score (HR: 1.19; P = 0.009) and nonapical lead position (HR: 2.17; P = 0.042) were all associated with death/transplantation. ConclusionsPPMv in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPMv cohort. Increased Vp, higher QRS z-score, and nonapical ventricular lead position are all associated with higher risk of adverse outcome and may be modifiable risk factors.

EP News: Pediatric and Congenital Electrophysiology

Mazzanti et al (JAMA Cardiol. 2022 Mar 30:e220219. PMID: 35353122) investigated 216 patients [55% female, median age 14 (IQR 9-30) years] with RYR2-CPVT treated with β-blockers only. The primary outcome was the first life-threatening arrhythmic event (LTAE), defined as sudden cardiac death, aborted cardiac arrest, or non-tolerated ventricular tachycardia, while taking β-blocker. During a mean follow-up of 9.4±7.5 years, 28/216 (13%) patients taking only β-blockers experienced an LTAE. In multivariable analysis, an LTAE before diagnosis (HR, 3.3; 95% CI, 1.2-8.9; P = .02), syncope before diagnosis (HR, 4.5; 95% CI, 1.8-11.1; P = .001) and a C-terminal domain variant (HR, 18.1; 95% CI, 4.1-80.8; P < .001) were each associated with an increased LTAE risk during β-blocker therapy.

EP News: Pediatric and Congenital Electrophysiology

Peltenburg et al (Circulation 2022;145:333–344, PMID 34874747) investigated the association between risk for arrhythmic events and type of β-blocker in 329 symptomatic children with catecholaminergic polymorphic ventricular tachycardia (CPVT). RYR2 variant–carrying symptomatic children (defined as syncope or sudden cardiac arrest before β-blocker initiation and start of β-blocker therapy <18 years of age) from both the International CPVT Registry and the PACES Pediatric CPVT Registry were enrolled.

Preliminary assessment of paediatric electrophysiology cardiac implantable electronic device resources around the world.

The Pediatric and Congenital Electrophysiology Society (PACES) is a global organisation committed to the care of children and adults with CHD and arrhythmias. To evaluate the global needs and potential inequities as it relates to cardiac implantable electronic devices. ARROW (Assessment of Rhythm Resources arOund the World) is an online survey about cardiac implantable electronic devices, sent electronically to physicians within the field of Cardiology, Pediatric Cardiology, Electrophysiology and Pediatric Electrophysiology. ARROW received 42 responders from 28 countries, 50% from low-/middle-income regions. The main differences between low-/middle- and high-income regions include availability of expertise on paediatric electrophysiology (50% versus 93%, p < 00.5) and possibility to perform invasive procedures (35% versus 93%, p < 0.005). Implant of devices in low-income areas relies significantly on patient's resources (71%). The follow-up of the devices is on the hands of paediatric cardiologist/electrophysiologist in higher resources centres (93% versus 50%, p < 0.05). The ARROW survey represents an initial assessment of the geographical characteristics in the field of Pediatric Electrophysiology. The next step is to make this "state of the art" more extensive to other aspects of the expertise. The relevance of collecting this data before the World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) in 2023 in Washington DC was emphasised in order to share the resulting information with the international community and set a plan of action to assist the development of arrhythmia services for children within developing regions of the world.

Primary Prevention Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease: Recommendations From Professional Societies.

Identifying adults with congenital heart disease (CHD) at high risk for sudden death who stand to benefit from primary prevention implantable cardioverter-defibrillators (ICDs) remains a major challenge. Inconsistencies among evidence-based recommendations by various professional societies can be a source of confusion to practicing clinicians. This article summarizes and compares recommendations from the Canadian Cardiovascular Society management guidelines on adults with CHD (2009), expert consensus from the Pediatric and Congenital Electrophysiology Society and Heart Rhythm Society (2014), European Society of Cardiology (ESC) guidelines on sudden death (2015), position statement from the European Heart Rhythm Association, Association for European Paediatric and Congenital Cardiology, and ESC working group on grown-up CHD (2018), and ESC guidelines on adult CHD (2020). Most recommendations are fundamentally similar, particularly with regards to: 1) recommending or considering primary prevention ICDs in adults with CHD and a systemic left ventricular ejection fraction ≤35%, biventricular physiology, and functional class II or III symptoms despite optimal medical therapy; 2) considering ICDs in those with unexplained syncope of suspected arrhythmic etiology and either advanced ventricular dysfunction or inducible sustained ventricular arrhythmias; and 3) considering ICDs in selected patients with tetralogy of Fallot and multiple risk factors for sudden death, such as left ventricular dysfunction, non-sustained ventricular tachycardia, QRS duration ≥180 ms, right ventricular scarring, or inducible sustained ventricular tachycardia. Areas of continued clinical uncertainty, which offer important opportunities for future research, include improving patient selection for primary prevention ICDs in the context of a systemic right ventricle, univentricular heart, or Eisenmenger syndrome.