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.

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 of a 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.

When Do Smartwatch Heart Rate Concerns in Children Indicate Arrhythmia?

OBJECTIVE: To determine the incidence and predictors of true arrhythmia in pediatric patients presenting with concerns about smartwatch cardiac data. STUDY DESIGN: Single-center, retrospective cohort study of children aged 10-18 years who had presented to a pediatric cardiology clinic between January 2018 and December 2021 with concerns related to smartwatch cardiac data. The primary study outcome was diagnosis of arrhythmia based on clinical evaluation or documentation of arrhythmia by clinical testing. RESULTS: There were 126 patients (mean age 15.6 ± 2.4 years) who presented with a smartwatch-based rhythm concern, with tachycardia in 89%. In all, 19 of 126 (15%) patients were diagnosed with true arrhythmia. The odds of a true arrhythmia diagnosis with symptoms vs no symptoms were 3.2 (95% CI 0.7-14.5), and with heart rate (HR) ≥190 beats/min vs HR <190 beats/min, it was 14.3 (95% CI 3.8-52.8). The positive predictive value of HR ≥190 beats/min and symptoms together to predict arrhythmia was only 39% (95% CI 28-52). The negative predictive value for arrhythmia having neither symptoms nor HR >190 was 95% (95% CI 75-99). CONCLUSION: The likelihood of a true arrhythmia in pediatric patients presenting with a smartwatch-based HR concern was low. Rarely, smartwatch electrograms or trend data were sufficient for arrhythmia diagnosis.

2023 HRS/EHRA/APHRS/LAHRS Expert Consensus Statement on Practical Management of the Remote Device Clinic.

Remote monitoring is beneficial for the management of patients with cardiovascular implantable electronic devices by impacting morbidity and mortality. With increasing numbers of patients using remote monitoring, keeping up with higher volume of remote monitoring transmissions creates challenges for device clinic staff. This international multidisciplinary document is intended to guide cardiac electrophysiologists, allied professionals, and hospital administrators in managing remote monitoring clinics. This includes guidance for remote monitoring clinic staffing, appropriate clinic workflows, patient education, and alert management. This expert consensus statement also addresses other topics such as communication of transmission results, use of third-party resources, manufacturer responsibilities, and programming concerns. The goal is to provide evidence-based recommendations impacting all aspects of remote monitoring services. Gaps in current knowledge and guidance for future research directions are also identified.

Variations in cardiac implantable electronic device surveillance and ancillary testing in the paediatric and congenital heart disease population: an international multi-centre survey from the Paediatric and Congenital Electrophysiology Society.

BACKGROUND: Expert guidance from scientific societies and regulatory agencies recommend a framework of principles for frequency of in-person evaluations and remote monitoring for patients with cardiac implantable electronic devices. However, there are limited data regarding adherence to recommendations among paediatric electrophysiologists, and there are no data regarding cardiac implantable electronic device-related ancillary testing. METHODS: To assess current clinical practices for cardiac implantable electronic device in-person evaluation, remote monitoring, and cardiac implantable electronic device-related ancillary testing, the Paediatric and Congenital Electrophysiology Society members were surveyed. The main outcome measures were variations in frequency of in person evaluation, frequency of remote monitoring, and cardiac implantable electronic device-related ancillary testing. RESULTS: All respondents performed in-person evaluation at least once a year, but <50% of respondents performed an in-person evaluation within 2 weeks of cardiac implantable electronic device implantation. Remote monitoring was performed every 3 months for pacemakers and implantable cardioverter defibrillators by 71 and 75% respondents, respectively. Follow-up echocardiography was performed every 2-3 years by 53% respondents for patients with >50% ventricular pacing. Majority of respondents (75%) did not perform either an exercise stress test or ambulatory Holter monitoring or chest X-ray (65%) after cardiac implantable electronic device implantation. CONCLUSION: This survey identified significant practice variations in cardiac implantable electronic device in- person evaluation, remote monitoring, and ancillary testing practices among paediatric electrophysiologists. Cardiac implantable electronic device management may be optimised by development of a paediatric-specific guidelines for follow-up and ancillary testing.

An International Multicenter Evaluation of Type 5 Long QT Syndrome: A Low Penetrant Primary Arrhythmic Condition.

BACKGROUND: Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic features associated with rare KCNE1 variants implicated in LQT5 was sought through an international multicenter collaboration. METHODS: Patients with either presumed autosomal dominant LQT5 (N = 229) or the recessive Type 2 Jervell and Lange-Nielsen syndrome (N = 19) were enrolled from 22 genetic arrhythmia clinics and 4 registries from 9 countries. KCNE1 variants were evaluated for ECG penetrance (defined as QTc >460 ms on presenting ECG) and genotype-phenotype segregation. Multivariable Cox regression was used to compare the associations between clinical and genetic variables with a composite primary outcome of definite arrhythmic events, including appropriate implantable cardioverter-defibrillator shocks, aborted cardiac arrest, and sudden cardiac death. RESULTS: A total of 32 distinct KCNE1 rare variants were identified in 89 probands and 140 genotype positive family members with presumed LQT5 and an additional 19 Type 2 Jervell and Lange-Nielsen syndrome patients. Among presumed LQT5 patients, the mean QTc on presenting ECG was significantly longer in probands (476.9±38.6 ms) compared with genotype positive family members (441.8±30.9 ms, P<0.001). ECG penetrance for heterozygous genotype positive family members was 20.7% (29/140). A definite arrhythmic event was experienced in 16.9% (15/89) of heterozygous probands in comparison with 1.4% (2/140) of family members (adjusted hazard ratio [HR] 11.6 [95% CI, 2.6-52.2]; P=0.001). Event incidence did not differ significantly for Type 2 Jervell and Lange-Nielsen syndrome patients relative to the overall heterozygous cohort (10.5% [2/19]; HR 1.7 [95% CI, 0.3-10.8], P=0.590). The cumulative prevalence of the 32 KCNE1 variants in the Genome Aggregation Database, which is a human database of exome and genome sequencing data from now over 140 000 individuals, was 238-fold greater than the anticipated prevalence of all LQT5 combined (0.238% vs 0.001%). CONCLUSIONS: The present study suggests that putative/confirmed loss-of-function KCNE1 variants predispose to QT prolongation, however, the low ECG penetrance observed suggests they do not manifest clinically in the majority of individuals, aligning with the mild phenotype observed for Type 2 Jervell and Lange-Nielsen syndrome patients.

The impact of CIEDs with automatic "wireless" remote monitoring on efficiency.

BACKGROUND: A benefit of automatically transmitting or "wireless" CIEDs (W-CIED) is the prompt detection of device malfunction and arrhythmias. We hypothesized that the use of W-CIEDs would improve the efficiency of remote monitoring by decreasing unnecessary CIED remote transmissions because of the automatic detection of abnormalities. OBJECTIVE: To compare the frequency of patient-initiated transmissions in patients with W-CIEDs versus non-wireless CIEDs (NW-CIED) at a single pediatric and congenital heart center. METHODS: Retrospective cohort study of patients with W-CIEDs followed over a 2-year period compared to a similar cohort of patients with NW-CIED. All CIED remote transmissions during were reviewed for indication and outcome. RESULTS: The W-CIED cohort had 87 patients; mean age 20 ± 13 years; NW-CIED cohort had 220 patients; mean age 22 ± (13) years. The mean number of symptomatic patient-initiated transmissions per patient was 0.93 ± 2.65 in the W-CIED cohort versus 0.39 ± 0.64 in the NW-CIED cohort (p ≤ .001). The mean number of asymptomatic patient-initiated transmission sent per patient in the W-CIED cohort was 1.86 ± 2.59 versus 0.81 ± 1.41 in the NW-CIED cohort (p ≤ .0001). Type of device, age, and presence of congenital heart disease were not significantly associated with the incidence of patient-initiated remote monitoring transmissions. CONCLUSIONS: The frequency of patient-initiated transmission was higher in the W-CIED cohort, contradictory to the study hypothesis. This may reflect a lack of patient understanding of the benefit or functionality of W-CIEDs and may be mitigated by education to both providers and patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

Frequency of CIED remote monitoring: A quality improvement follow-up study.

BACKGROUND: Based on the findings of a prior study of CIED (Cardiac Implantable Electrical Device) remote monitoring (RM) frequency at the same center, the University of Michigan Congenital Heart Center (UMCHC) instituted a quality improvement (QI) change to reduce the frequency of routine CIED RM from every 2 months to every 3 months. The objective of this study is to determine the impact of this QI initiative to reduce workload without compromising patient care. METHODS: This is a single-center, retrospective cohort study of all UMCHC patients with CIEDs followed via Medtronic CareLink CIED remote monitoring system from July 2015 to June 2017, after the QI change in 2014. The primary outcome was success of transition to new monitoring schedule. Secondary outcomes included complications, incidence of actionable events (AES), patient compliance, and change in workload. Outcomes were compared to the prior study. RESULTS: There were 325 patients (mean age was 24  ±  14 years) included, of who 293 (90%) completely transitioned to the new RM schedule. During the study period, 96 transmissions included AES (4% of total), of which 50 (52%) were asymptomatic and discovered on routine monitoring. No patient experienced a complication attributable to decreased RM frequency. The mean number of interrogations decreased by 1.6 per patient over the 2-year period compared to prior study. CONCLUSIONS: This study demonstrated successful implementation of a QI initiative to reduce CIED monitoring frequency at a single center with no patient adverse events. The intervention reduced workload and potentially improved patient compliance with routine RM.

Variation in Pediatric Post-Ablation Care: A Survey of the Pediatric and Congenital Electrophysiology Society (PACES).

Although catheter ablation is a standard treatment for pediatric arrhythmias, there are no consensus guidelines for follow-up care. This study describes the variation in post-ablation practices identified through a survey of the pediatric and congenital electrophysiology society (PACES). Pediatric and congenital electrophysiology society members were invited to participate in an online survey of post-ablation practices in September 2014. Survey questions targeted routine post-ablation practices for three common arrhythmia substrates: atrioventricular nodal reentry tachycardia, concealed accessory pathways (AP), and manifest APs. Significant practice variation was defined as <90% concordance among respondents. There were 70 respondents from 67 centers, 29 (41%) in practice for <10 years. Uniform practices included aspirin after left side ablation by 65 (93%), immediate post-procedure ECG by 63 (90%), and performance of outpatient follow-up in 69 (99%) including ECG in 97-100% depending on substrate. The majority, 57 (81%), have standardized follow-up independent of substrate. Post-procedural observation is highly variable, with 25 (36%) discharging patients on the day of ablation, 22 (33%) observing patients in hospital overnight, and 21 (30%) basing hospitalization on pre-defined criteria. Immediate post-procedure echo is performed after all ablations in only 16 (23%). Discharge from outpatient care occurs at a median time of 12 months for each arrhythmia substrate. Common post-ablation practices are evident among pediatric electrophysiologists. However, they report significant variation in post-procedure monitoring practices and testing. The rationale for these variances, and their impact on costs and outcomes, should be defined.

Behavioural and emotional implications of implantable cardioverter-defibrillators in the young and in athletes.

Despite the life-saving capabilities of implantable cardioverter-defibrillators, they may have implications on behavioural and emotional well-being and have been shown to negatively affect patients' psychosocial functioning. Children and CHD patients with these devices are at higher risk for complications, and therefore may have higher risk of psychosocial dysfunction including depression, anxiety, and a decrease in overall quality of life. In addition, these patients may be restricted from activities, which may also contribute to psychosocial dysfunction. Recommendations published in 2015 support a more liberal approach to athletic participation in this patient population compared with previous guidelines. Approaches to limit psychosocial dysfunction include education, minimisation of shocks, and psychosocial therapy. Psychosocial dysfunction should be assessed at each clinic visit, and information regarding intervention should be provided to patients and their families as needed. Psychosocial dysfunction may be debilitating, and healthcare providers should facilitate and support normal psychosocial function by offering resources as needed.

Implantable Cardioverter Defibrillator Outcomes in Pediatric and Congenital Heart Disease: Time to System Revision.

BACKGROUND: Implantable cardioverter defibrillators (ICDs) are intended to prevent sudden cardiac death yet also impose a risk of morbidity. This study describes the outcomes of ICDs in a pediatric and congenital heart disease (CHD) population from a single center. METHODS: Retrospective cohort study of all patients with an ICD followed at the University of Michigan Congenital Heart Center from 2005-2013. The primary outcome was ICD system revision for any reason excluding routine generator change for battery depletion. RESULTS: There were 191 ICD systems in 131 patients, including 57 with CHD, 24 with hypertrophic cardiomyopathy, and 45 with structurally normal hearts. Median age was 16 years at initial implant. Total follow-up was 850 patient-years; median 4.9 years/patient. There were 43 (33%) patients who required 60 ICD revisions; 70 revisions/1,000 patient-years of follow-up. Revisions included 25 lead extractions with replacement, 21 lead additions, five lead repositions, and four full system revisions. Kaplan-Meier (K-M) median time to appropriate shock was similar to the median time to system revision. K-M time to system revision was significantly affected by recalled lead performance. CONCLUSIONS: The need for ICD system revision is high in this pediatric and CHD population and occurs at a rate similar to the rate of receiving appropriate therapy. These results highlight the need for judicious implant criteria and improved device longevity.

Nurse Practitioner Delivered Virtual Care in a Pediatric Specialty Clinic.

INTRODUCTION: Virtual care in pediatric specialty practice has become widely used to increase access to care, one in which nurse practitioners (NPs) can play a vital role. We sought to evaluate the outcomes of virtual care provided by pediatric cardiology and electrophysiology (EP) NPs for pediatric and congenital heart disease patients. METHOD: Retrospective review of all virtual care visits performed by pediatric EP NPs between October 2019 and October 2021 at a single tertiary care center. RESULTS: NPs delivered virtual care for 287 pediatric EP evaluations for 276 patients; 132 (45%) independently, with the remaining 155 collaborating with an electrophysiologist as a shared visit. The mean age was 15.2 ± 8.9 years. DISCUSSION: NPs performed a significant subset of these visits independently and were a vital part of all visits in this study. The role of the NP in specialty pediatric virtual care should continue to be supported and advanced.

Multicenter retrospective evaluation of magnetic resonance imaging in pediatric and congenital heart disease patients with cardiac implantable electronic devices.

BACKGROUND: Guidelines addressing magnetic resonance imaging (MRI) in patients with cardiac implantable electronic devices (CIEDs) provide algorithms for imaging pediatric and congenital heart disease (CHD) patients. Guideline acceptance varies by institution. Guidelines also do not support routine MRI scans in patients with epicardial or abandoned leads, common in pediatric and CHD patients. OBJECTIVE: The purpose of this study was to determine the incidence of MRI-related complications in pediatric and CHD patients with CIEDs, including epicardial and/or abandoned leads. METHODS: A multicenter retrospective review included patients with CIEDs who underwent any MRI between 2007 and 2022 at congenital cardiac centers. The primary outcome was any patient adverse event or clinically significant CIED change after MRI, defined as pacing lead capture threshold increase >0.5 V with output change, P- or R- wave amplitude decrease >50% with sensitivity change, or impedance change >50%. RESULTS: Across 14 institutions, 314 patients (median age 18.8 [1.3; 31.4] years) underwent 389 MRIs. There were 288 pacemakers (74%) and 87 implantable cardioverter-defibrillators (22%); 52% contained epicardial leads, and 14 (4%) were abandoned leads only. Symptoms or CIED changes occurred in 4.9% of MRI scans (6.1% of patients). On 9 occasions (2%), warmth or pain occurred. Pacing capture threshold or lead impedance changes occurred in 1.4% and 2.0% of CIEDs post-MRI and at follow-up. CONCLUSION: Our data provide evidence that MRIs can be performed in pediatric and CHD patients with CIEDs, including non-MRI-conditional CIEDs and epicardial and/or abandoned leads, with rare minor symptoms or CIED changes but no other complications.

2023 HRS/EHRA/APHRS/LAHRS expert consensus statement on practical management of the remote device clinic.

Remote monitoring is beneficial for the management of patients with cardiovascular implantable electronic devices by impacting morbidity and mortality. With increasing numbers of patients using remote monitoring, keeping up with higher volume of remote monitoring transmissions creates challenges for device clinic staff. This international multidisciplinary document is intended to guide cardiac electrophysiologists, allied professionals, and hospital administrators in managing remote monitoring clinics. This includes guidance for remote monitoring clinic staffing, appropriate clinic workflows, patient education, and alert management. This expert consensus statement also addresses other topics such as communication of transmission results, use of third-party resources, manufacturer responsibilities, and programming concerns. The goal is to provide evidence-based recommendations impacting all aspects of remote monitoring services. Gaps in current knowledge and guidance for future research directions are also identified.

2023 HRS/EHRA/APHRS/LAHRS Expert Consensus Statement on Practical Management of the Remote Device Clinic.

Remote monitoring is beneficial for the management of patients with cardiovascular implantable electronic devices by impacting morbidity and mortality. With increasing numbers of patients using remote monitoring, keeping up with higher volume of remote monitoring transmissions creates challenges for device clinic staff. This international multidisciplinary document is intended to guide cardiac electrophysiologists, allied professionals, and hospital administrators in managing remote monitoring clinics. This includes guidance for remote monitoring clinic staffing, appropriate clinic workflows, patient education, and alert management. This expert consensus statement also addresses other topics such as communication of transmission results, use of third-party resources, manufacturer responsibilities, and programming concerns. The goal is to provide evidence-based recommendations impacting all aspects of remote monitoring services. Gaps in current knowledge and guidance for future research directions are also identified.

A Survey of Immunization Practices in Patients With Congenital Heart Disease.

Background: Congenital heart disease, the most common congenital anomaly, often presents in neonates. Because of perceived risks, health care providers may consider deferring immunizations in this population. We sought to understand the perceived risk of immunizations in those providing health care to children with particular heart conditions. Methods: A survey, which included 6 hypothetical scenarios assessing immunization recommendations, was distributed internationally to relevant health care providers, and responses were compared between the different scenarios. Results: Majority of responses (n = 142) were from paediatric cardiologists (n = 98; 69%) and nurse practitioners (n = 27; 19%) located in the United States (n = 77; 54%) or Canada (n = 53; 37%) working in academic teaching hospitals (n = 133; 93.7%). Most favoured vaccinations (n = 107; 75.4%) and less likely to proceed with the first immunization in infants with structural heart disease compared with channelopathy (risk ratio: 0.80, confidence interval: 0.73-0.87; P < 0.001). Only 40% would proceed with immunization as normal in an infant with manifest Brugada type I electrocardiogram. Special precautions after the immunization included longer duration of observation (19%) and administering prophylactic antipyretic medication (92%). Conclusions: Respondents were 20% more likely to defer immunizations in the presence of treatable structural heart disease as compared with channelopathy despite the lack of evidence supporting deferring immunizations in children with structural heart disease. Most were cautious in their response to the scenario involving Brugada syndrome, indicating awareness of the risk of haemodynamic instability in the event of a fever. The majority of respondents still strongly recommend immunizations in this population as the benefits outweigh the potential for adverse events.

Contexte: La cardiopathie congénitale ­ l'anomalie congénitale la plus courante ­ est souvent observée chez les nouveau-nés. En raison des risques perçus, les dispensateurs de soins de santé peuvent parfois envisager de reporter la vaccination chez ces patients. Notre but était de comprendre le risque perçu à l'égard de la vaccination par les dispensateurs de soins de santé traitant des enfants atteints de certaines cardiopathies. Méthodologie: Un sondage comprenant six scénarios hypothétiques visant à évaluer les recommandations de vaccination a été distribué à des dispensateurs de soins de santé pertinents dans différents pays, et leurs réponses pour les différents scénarios ont été comparées. Résultats: La majorité des répondants (n = 142) étaient des cardiologues pédiatriques (n = 98; 69 %) ou des infirmières praticiennes (n = 27; 19 %) des États-Unis (n = 77; 54 %) ou du Canada (n = 53; 37 %) travaillant dans des hôpitaux universitaires (n = 133; 93,7 %). La plupart d'entre eux étaient en faveur de la vaccination (n = 107; 75,4 %), bien que moins enclins à administrer un premier vaccin à des nourrissons présentant une cardiopathie structurelle comparativement à une canalopathie (rapport des risques : 0,80, intervalle de confiance : 0,73-0,87; p < 0,001). Or, seulement 40 % d'entre eux vaccineraient de façon normale un nourrisson présentant un syndrome de Brugada de type 1 à l'ECG. Les précautions particulières prises après la vaccination comprenaient une période d'observation plus longue (19 %) et l'administration d'un antipyrétique à des fins prophylactiques (92 %). Conclusions: À la lumière des réponses obtenues, la probabilité de report de la vaccination était 20 % plus élevée en présence d'une cardiopathie structurelle traitable comparativement à une canalopathie, malgré le manque de données probantes justifiant ce report chez les enfants atteints d'une cardiopathie structurelle. La plupart des répondants ont répondu de façon prudente au scénario du syndrome de Brugada en évoquant un risque d'instabilité hémodynamique en cas de fièvre. La majorité d'entre eux recommandent quand même fortement la vaccination chez ces patients, car les bienfaits escomptés l'emportent sur les risques d'effets indésirables.