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.

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.

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.

Institutional experience of healthy pediatric patients presenting with atrial fibrillation who had an electrophysiology study.

INTRODUCTION: Atrial fibrillation (AF) is a very common tachyarrhythmia with increasing prevalence with age, but uncommon in the pediatric population. Understanding that AF increases comorbidities make the need for investigation and potential elimination of alternate etiologies in pediatric AF patients critical. The objective of this study was to review our institutional data and compare our findings with previously documented adult AF risk factors to pediatric patients while also identifying which patients had alternate electrophysiology diagnoses amenable to transcatheter ablation. METHODS: A retrospective chart review was performed identifying AF patients who were less than 21 years old, had no significant congenital cardiovascular anomalies, a documented episode of AF on electrocardiogram and underwent invasive electrophysiology study (EPS). RESULTS: Nineteen patients were identified over a 9-year period of time finding a male predominance (74%), the average age of 14.95 ± 4.17 years, the average weight of 78.5 ± 31.4 kg, and average body mass index of 26.8 ± 6.87 kg/m2 . Preprocedural left atrial volumes made on echocardiograms demonstrated a mean of 33.96 ± 16.35 mL/m2 (Z-scores -0.81 ± 1.50), indicating no dilation. Five of nineteen patients (26%) had additional electrophysiologic diagnoses during EPS, including atrioventricular reentrant tachycardia (n = 2, 10%) and atrioventricular nodal reentrant tachycardia (n = 3, 16%). Four patients underwent successful ablation with no documented or clinical AF recurrence. CONCLUSIONS: Adult risk factors of male predominance and obesity were seen in pediatric AF patients, while left atrial enlargement was not. Twenty-one percent of the pediatric AF patients who had additional electrophysiologic substrates and successful ablations resulted in no further clinical episodes of AF. This suggests that pediatric patients presenting with AF might benefit from an EPS as part of a complete evaluation.

Photo-reorganization of 3-alkoxy-6-chloro-2-(benzo[b]thiophen-2-yl)-4H-chromen-4-ones: a green and convenient synthesis of angular pentacyclics.

Photo-reorganization of 3-alkoxy-6-chloro-2-(benzo[b]thiophen-2-yl)-4H-chromen-4-ones in methanol with Pyrex filtered UV-light from a medium pressure 125 W Hg-vapor lamp led to the formation of angular pentacyclic compounds (dihydro and aromatic products) along with some rearranged chromenones where the product(s) distribution depended upon the structure of 3-alkoxy groups (methoxy, ethoxy, allyloxy and benzyloxy). The phenyl moiety in the 3-benzyloxy group had a profound effect on the dihydro product(s) formation as the latter was in high yield when the alkoxy group was benzyloxy followed by allyloxy, ethoxy and methoxy groups. The present photochemical study represents a general method for the synthesis of some angular pentacyclic - benzothiophene fused xanthenone derivatives in a single step without using any specific and toxic reagent. The structures of the new organic scaffolds obtained were established by their spectral data (UV, IR and NMR).

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest.

OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014. RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). CONCLUSION: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

Phototransformation of 3-alkoxychromenones: regioselective photocyclisation and dealkoxylation.

The phototransformation of some 2-(3-methoxyphenyl)-4H-chromen-4-ones bearing a propynyloxy moiety at the 3-position has been described. On photolysis with pyrex-filtered UV light from a Hg lamp (125 W), these chromenones produced a major amount of 5-ethynyl-2-methoxy-6-oxa-benzo[5,6-c]xanthen-7-ones consisting of an exotic tetracyclic scaffold. These photoproducts have been envisioned to be produced through regioselective ring closure at the 6'-position of the 2-(3'-methoxy)phenyl moiety of the initially formed 1,4-biradical via a γ-H abstraction mechanism. No product whatsoever was observed through ring closure at the 2'-position. This behaviour has been found to be in accordance with the directive influence observed in free radical aromatic substitutions. This regioselective photocyclisation is further supported by calculations made from 3D structures (MM2 program). In addition, during the irradiation of these substrates, 2-(3-methoxyphenyl)-4H-chromen-4-ones were also realised through dealkoxylation. The structures of the substrates and photoproduct(s) have been determined by their spectroscopic (IR, NMR, mass spectrometry) studies.

Absorption and Fluorescent Studies of 3-Hydroxychromones.

The synthesis and spectral studies of variously substituted 3-hydroxychromones have been carried out. A key relationship between the structural motif of synthesized 3-hydroxychromones (3-HCs) and their fluorescent properties was found. The chromones substituted with electron-donating group at 4'-position expressed the red shift of the N(*) and T(*) band and also exhibited the increased fluorescent intensity ratio while the chromones with electron-withdrawing group showed the blue shift of the N(*) and T(*) band. Therefore, these 3-HCs may behave as the possible fluorescent probes.

Radiation Exposure, Training, and Safety in Cardiology.

Exposure to ionizing radiation is an inherent occupational health hazard in clinical cardiology. Health risks have been reported previously, including predilection to cancer. In addition, orthopedic injury due to prolonged wearing of heavy protective lead aprons, which are mandatory to reduce radiation risk, have been extensively documented. Cardiology as a specialty has grown with rising volumes of increasingly complex procedures. This includes electrophysiological, coronary, and structural intervention, advanced heart failure/transplant management, and diagnostic imaging. Both the operator as well imaging specialists are exposed to radiation, particularly in structural interventions where interventional cardiologists and structural imagers work closely. Increasingly, women interested in cardiology may deselect the field due to radiation concerns. This expert document highlights the risks of radiation exposure in cardiology, including practical tips within various subspecialty fields such as interventional/structural cardiology, electrophysiology, imaging, advanced heart failure, and pediatric cardiology.

Antithrombotic Therapy for Patients Undergoing Cardiac Electrophysiological and Interventional Procedures: JACC State-of-the-Art Review.

Electrophysiological and interventional procedures have been increasingly used to reduce morbidity and mortality in patients experiencing cardiovascular diseases. Although antithrombotic therapies are critical to reduce the risk of stroke or other thromboembolic events, they can nonetheless increase the bleeding hazard. This is even more true in an aging population undergoing cardiac procedures in which the combination of oral anticoagulants and antiplatelet therapies would further increase the hemorrhagic risk. Hence, the timing, dose, and combination of antithrombotic therapies should be carefully chosen in each case. However, the maze of society guidelines and consensus documents published so far have progressively led to a hazier scenario in this setting. Aim of this review is to provide-in a single document-a quick, evidenced-based practical summary of the antithrombotic approaches used in different cardiac electrophysiology and interventional procedures to guide the busy clinician and the cardiac proceduralist in their everyday practice.

Conduction System Pacing for Patients with Congenital Heart Disease.

For patients with congenital heart disease (CHD), chronic ventricular pacing may lead to progressive cardiomyopathy owing to electromechanical dyssynchrony. Cardiac conduction system pacing (CSP) has been proposed as a physiologic pacing strategy-directly engaging the His-Purkinje system and preserving electromechanical synchrony. CSP may be indicated for a wide variety of children and adults with CHD and has emerged as an important tool in the armamentarium for cardiac implantable electronic device operators. This review provides the rationale, background, and supportive evidence for CSP in patients with CHD and discusses implant strategies and outcomes in the context of dominant ventricular morphologic categories.

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.

Implementation and early experience of a pediatric electrophysiology telehealth program.

Background: Telehealth (TH) visits have been growing with exponential increased utilization during the COVID-19 pandemic. The aim of this manuscript is to describe the implementation and early experience of a pediatric electrophysiology (EP) TH program implemented during the pandemic, assessing patient satisfaction, patient equity and inclusion (measured by geographical outreach), and sustainability. Methods: A retrospective chart review study was performed and data were collected from the medical record, including demographic, testing, and billing data from scheduled TH encounters between March and August 2020 of a single pediatric EP group in the Midwest. Patients were called to complete satisfaction surveys. Results: Patients with diverse pathologies were seen in TH, with supraventricular/atrial tachycardias (n = 41, 35%) and inherited arrhythmia syndromes (n = 23, 20%) being most common. The mean distance from clinic was 95 miles (range 2.8-320 miles), with 43% of patients living more than 100 miles away from clinic. A total of 172 tests were performed previsit (n = 102, 59%), during the visit (n = 17, 10%), or postvisit (n = 53, 31%), including 15 EP studies. Time-based Current Procedural Terminology codes were predominantly used for billing purposes (n = 92, 78%). There was generation of work relative value units (wRVU) for visits (220.5 wRVU) and testing (325.1 wRVU). Survey data demonstrated that 98% of patients were satisfied with their telehealth appointment and 99% had a clear understanding of their diagnosis. Conclusion: Pediatric EP TH clinics can provide care for a geographically and pathologically heterogeneous group of patients who had positive attitudes toward TH. Our study shows significant downstream testing and subsequent wRVU generation, suggesting financial sustainability.

A Single-center Experience Comparing First- Versus Second-generation Insertable Cardiac Monitors in Pediatric Patients.

Insertable cardiac monitors (ICMs) have undergone advancements in size and functionality over the past decade, resulting in the introduction of small, easily insertable devices capable of long-term remote monitoring. We define first-generation ICMs as implantable cardiac monitoring devices that require an incision and surgical creation of a subcutaneous pocket and second-generation ICMs as devices implanted using a custom-made tool for subcutaneous insertion, respectively. The aim of this study was to understand the differences between first- and second-generation pediatric ICM implants, implant indications, and time to diagnosis. We performed a retrospective, single-center chart review of patients who underwent ICM implantation from 2009-2019, spanning a 5-year course of first-generation ICM implantations and 5-year course of second-generation ICM implantations. Demographic data, past medical history, implant indication, and time to diagnosis were obtained. A total of 208 patients were identified over the 10-year time period, including 38 (18%) who underwent implantation with a first-generation device and 170 (82%) who underwent implantation with a second-generation device. Implant indications for first-generation ICMs included syncope (71%), palpitations (16%), inherited arrhythmia syndrome (IAS) management (5%), and premature ventricular contractions/ventricular tachycardia (VT) (8%); implant indications for second-generation ICMs included syncope (48%), palpitations (19%), IAS management (40%), premature ventricular contractions/VT (11%), atrial fibrillation (2%), tachycardia (3%), and heart block (0.5%). The average time to diagnosis was 38 weeks for patients with first-generation devices and 55 weeks for those with second-generation devices. With innovations in ICM technologies, there are expanding indications for ICM implantation in pediatric patients for long-term monitoring, specifically regarding the management of IAS patients.

Approach to Wide Complex Tachycardia in Paediatric Patients.

Wide complex tachycardia (WCT) is an infrequently encountered condition in paediatric patients and may be due to a variety of causes including supraventricular tachycardia with aberrant conduction, ventricular activation via an accessory pathway, ventricular pacing, or ventricular tachycardia. Immediate tachycardia termination is required in haemodynamically unstable patients. After stabilization or in those with haemodynamically tolerated WCT, a careful review of electrocardiographic tracings and diagnostic manoeuvres are essential to help elucidate the cause. Subacute and chronic management for WCT will depend on the underlying cause as well as features of the patient and the tachycardia presentation. This article will review the epidemiology, potential causes, and management of WCT in children. A detailed review of the pathophysiology, differential diagnosis, and diagnostic and treatment options is provided to enable the reader to develop a practical approach to managing this condition in young patients.

La tachycardie à complexes QRS larges est rare en pédiatrie et peut avoir diverses causes, notamment une tachycardie supraventriculaire avec trouble de la conduction, l'activation ventriculaire par une voie accessoire, une stimulation ventriculaire ou une tachycardie ventriculaire. La suppression immédiate de la tachycardie est primordiale lorsque l'état hémodynamique du patient est instable. Une fois l'état du patient stabilisé, ou en cas de tachycardie à complexes QRS larges tolérée sur le plan hémodynamique, l'examen minutieux des tracés électrocardiographiques et des manœuvres diagnostiques est crucial pour en élucider la cause. La prise en charge des cas subaigus et chroniques de tachycardie à complexes QRS larges dépend de sa cause sous-jacente ainsi que des caractéristiques du patient et du tableau clinique de la tachycardie. Cet article porte sur l'épidémiologie, les causes possibles et la prise en charge de la tachycardie à complexes QRS larges chez les enfants. Un examen approfondi de la physiopathologie, du diagnostic différentiel et des options diagnostiques et thérapeutiques est présenté pour permettre au lecteur d'élaborer une approche pratique pour la prise en charge de cette affection chez leurs jeunes patients.