Premature ventricular complexes: Assessing burden density in a large national cohort to better define optimal ECG monitoring duration.

BACKGROUND: Premature ventricular contraction (PVC) burden is a risk factor for heart failure and cardiovascular death in patients with structural heart disease. Long-term electrocardiographic monitoring can have a significant impact on PVC burden evaluation by further defining PVC distribution patterns. OBJECTIVE: This study aimed to ascertain the optimal duration of electrocardiographic monitoring to characterize PVC burden and to understand clinical characteristics associated with frequent PVCs and nonsustained ventricular tachycardia in a large US cohort. METHODS: Commercial data (iRhythm's Zio patch) from June 2011 to April 2022 were analyzed. Inclusion criteria were age >18 years, PVC burden ≥5%, and wear period ≥13 days. PVC burden cutoffs were determined on the basis of AHA/ACC/HRS guidelines for very frequent PVCs (10,000-20,000 during 24 hours). Patients were assigned to categories by PVC densities: low, <10%; moderate, 10% to <20%; and high, ≥20%. Mean measured error was assessed at baseline and daily until the wear period's end for overall PVC burden and different PVC densities. RESULTS: Analysis of 106,705 patch monitors revealed a study population with mean age of 70.6 ± 14.6 years (33.6% female). PVC burden was higher in male patients and those >65 years of age. PVC burden mean error decreased from 2.9% at 24 hours to 1.3% at 7 days and 0.7% at 10 days. Number of ventricular tachycardia episodes per patient increased with increasing PVC burden (P < .0001). CONCLUSION: Extending ambulatory monitoring beyond 24 hours to 7 days or more improves accuracy of assessing PVC burden. Ventricular tachycardia frequency and duration vary by initial PVC density, highlighting the need for prolonged cardiac monitoring.

Prevalence of Subclinical Atrial Fibrillation in Heart Failure With Preserved Ejection Fraction.

BACKGROUND: Atrial fibrillation (AF) is common in patients with heart failure with preserved ejection fraction (HFpEF) and is associated with poorer clinical outcomes. The prevalence of subclinical AF in patients with HFpEF remains unknown. OBJECTIVES: The aim of this study was to determine whether subclinical AF was more prevalent in individuals with HFpEF than in individuals without histories of heart failure (HF). METHODS: Patients with HFpEF with no prior diagnoses of AF were screened for subclinical AF, and the prevalence of subclinical AF was compared with that among control subjects without HF drawn from MESA (Multi-Ethnic Study of Atherosclerosis) who underwent the same electrocardiographic monitoring. Multivariable logistic regression was used to adjust for demographic and clinical comorbidities. RESULTS: Ninety patients with HFpEF and 1,230 MESA participants were included. Patients with HFpEF were younger (median age 69 years [Q1-Q3: 63-76 years] vs 72 years [Q1-Q3: 66-80 years]; P = 0.02), more obese (median body mass index 36 kg/m2 [Q1-Q3: 30-45 kg/m2] vs 27 kg/m2 [Q1-Q3: 24-30 kg/m2]; P < 0.001), and more likely to have diabetes (34% vs 21%; P = 0.01). The prevalence of subclinical AF was 8.9% in patients with HFpEF and 4.1% in non-HF participants. After multivariable adjustment for age, sex, race, body mass index, diabetes, smoking, and total analyzable time on electrocardiographic monitor, there was a significantly higher odds of subclinical AF in patients with HFpEF compared with MESA (OR: 3.01; 95% CI: 1.13-7.99; P = 0.03). CONCLUSIONS: Patients with HFpEF had a higher prevalence of subclinical AF than participants without HF from a community-based study. Screening for atrial arrhythmias may be appropriate among patients with HFpEF for timely initiation of thromboembolic prophylaxis and may identify individuals at greater risk for clinical decompensation.

Can 24 h of ambulatory ECG be used to triage patients to extended monitoring?

BACKGROUND: Access to long-term ambulatory recording to detect atrial fibrillation (AF) is limited for economical and practical reasons. We aimed to determine whether 24 h ECG (24hECG) data can predict AF detection on extended cardiac monitoring. METHODS: We included all US patients from 2020, aged 17-100 years, who were monitored for 2-30 days using the PocketECG device (MEDICALgorithmics), without AF ≥30 s on the first day (n = 18,220, mean age 64.4 years, 42.4% male). The population was randomly split into equal training and testing datasets. A Lasso model was used to predict AF episodes ≥30 s occurring on days 2-30. RESULTS: The final model included maximum heart rate, number of premature atrial complexes (PACs), fastest rate during PAC couplets and triplets, fastest rate during premature ventricular couplets and number of ventricular tachycardia runs ≥4 beats, and had good discrimination (ROC statistic 0.7497, 95% CI 0.7336-0.7659) in the testing dataset. Inclusion of age and sex did not improve discrimination. A model based only on age and sex had substantially poorer discrimination, ROC statistic 0.6542 (95% CI 0.6364-0.6720). The prevalence of observed AF in the testing dataset increased by quintile of predicted risk: 0.4% in Q1, 2.7% in Q2, 6.2% in Q3, 11.4% in Q4, and 15.9% in Q5. In Q1, the negative predictive value for AF was 99.6%. CONCLUSION: By using 24hECG data, long-term monitoring for AF can safely be avoided in 20% of an unselected patient population whereas an overall risk of 9% in the remaining 80% of the population warrants repeated or extended monitoring.

Low-Power Long-Term Ambulatory Electrocardiography Monitor of Three Leads with Beat-to-Beat Heart Rate Measurement in Real Time.

A low-power long-term ambulatory ECG monitor was developed for the acquisition, storage and processing of three simultaneous leads DI, aVF and V2 with a beat-to-beat heart rate measurement in real time. It provides long-term continuous ECG recordings until 84 h. The monitor uses a QRS complex detection algorithm based on the continuous wavelet transform with splines, which automatically selects the scale for the analysis of ECG records with different sampling frequencies. It includes a lead-off detection to continuously monitor the electrode connections and a real-time system of visual and acoustic alarms to alert users of abnormal conditions in its operation. The monitor presented is based in an ADS1294 analogue front end with four channels, 24-bit analog-to-digital converters and programmable gain amplifiers, a low-power dual-core ESP32 microcontroller, a microSD memory for data storage in a range of 4 GB to 32 GB and a 1.4 in thin-film transistor liquid crystal display (LCD) variant with a resolution of 128 × 128 pixels. It has programmable sampling rates of 250, 500 and 1000 Hz; a bandwidth of 0 Hz to 50% of the selected sampling rate; a CMRR of -105 dB; an input margin of ±2.4 V; a resolution of 286 nV; and a current consumption of 50 mA for an average battery life of 84 h. The ambulatory ECG monitor was evaluated with the commercial data-acquisition system BIOPAC MP36 and its module for ECG LABEL SS2LB, simultaneously comparing the morphologies of two ECG records and obtaining a correlation of 91.78%. For the QRS detection in real time, the implemented algorithm had an error less than 5%. The developed ambulatory ECG monitor can be used for the analysis of the dynamics of the heart rate variability in long-term ECG records and for the development of one's own databases of ECG recordings of normal subjects and patients with cardiovascular and noncardiovascular diseases.

Ventricular tachycardia risk prediction with an abbreviated duration mobile cardiac telemetry.

Background: Ventricular tachycardia (VT) occurs intermittently, unpredictably, and has potentially lethal consequences. Objective: Our aim was to derive a risk prediction model for VT episodes ≥10 beats detected on 30-day mobile cardiac telemetry based on the first 24 hours of the recording. Methods: We included patients who were monitored for 2 to 30 days in the United States using full-disclosure mobile cardiac telemetry, without any VT episode ≥10 beats on the first full recording day. An elastic net prediction model was derived for the outcome of VT ≥10 beats on monitoring days 2 to 30. Potential predictors included age, sex, and electrocardiographic data from the first 24 hours: heart rate; premature atrial and ventricular complexes occurring as singlets, couplets, triplets, and runs; and the fastest rate for each event. The population was randomly split into training (70%) and testing (30%) samples. Results: In a population of 19,781 patients (mean age 65.3 ± 17.1 years, 43.5% men), with a median recording time of 18.6 ± 9.6 days, 1510 patients had at least 1 VT ≥10 beats. The prediction model had good discrimination in the testing sample (area under the receiver-operating characteristic curve 0.7584, 95% confidence interval 0.7340-0.7829). A model excluding age and sex had an equally good discrimination (area under the receiver-operating characteristic curve 0.7579, 95% confidence interval 0.7332-0.7825). In the top quintile of the score, more than 1 in 5 patients had a VT ≥10 beats, while the bottom quintile had a 98.2% negative predictive value. Conclusion: Our model can predict risk of VT ≥10 beats in the near term using variables derived from 24-hour electrocardiography, and could be used to triage patients to extended monitoring.

Comparison of Medical Treatments According to the Characteristics of Idiopathic Premature Ventricular Contractions: Beta-blockers or Calcium Channel Blockers?

Objective: Premature ventricular contractions (PVCs) are a common arrhythmic condition. The first approach in patients with symptomatic and frequent PVC is medical treatment, primarily beta-blockers (BB) or calcium channel blockers (CCB), but it is still unclear which of the two should be chosen. This study investigated which drug treatment would be beneficial according to patient and electrocardiography (ECG) characteristics in patients with idiopathic PVC. Methods: We retrospectively analyzed 156 patients with PVC who came to the cardiology outpatient clinic. Seventy-one patients were responsive to BB, and 85 were responsive to CCB. Their demographic and ECG characteristics were compared. Results: The male ratio was higher (p<0.001), and the left ventricular ejection fraction was lower in BB responders than in CCB responders (p<0.001). Although the mean heart rate was higher in BB responders (p<0.001), the initial PVC burden was lower in BB responders than in CCB responders (p<0.001). The PVC QRS duration was longer in BB responders than in CCB responders (p<0.001). Similarly, the coupling interval variability was higher in BB responders (p=0.006). Conclusions: The evaluation of clinical and ECG parameters in patients with frequent idiopathic PVCs may determine whether BBs or CCBs should be chosen as initial treatment. Further prospective studies are needed to verify our findings and establish their clinical applicability.

Development and Optimization of Silicon-Dioxide-Coated Capacitive Electrode for Ambulatory ECG Measurement System.

This paper presents a silicon-dioxide-coated capacitive electrode system for an ambulatory electrocardiogram (ECG). The electrode was coated with a nano-leveled (287 nm) silicon dioxide layer which has a very high resistance of over 200 MΩ. Due to this high resistance, the electrode can be defined as only a capacitor without a resistive characteristic. This distinct capacitive characteristic of the electrode brings a simplified circuit analysis to achieve the development of a high-quality ambulatory ECG system. The 240 um thickness electrode was composed of a stainless-steel sheet layer for sensing, a polyimide electrical insulation layer, and a copper sheet connected with the ground to block any electrical noises generated from the back side of the structure. Six different diameter electrodes were prepared to optimize ECG signals in ambulatory environment, such as the amplitude of the QRS complex, amplitude of electromagnetic interference (EMI), and baseline wandering of the ECG signals. By combining the experimental results, optimal ambulatory ECG signals were obtained with electrodes that have a diameter from 1 to 3 cm. Moreover, we achieved high-quality ECG signals in a sweating simulation environment with 2 cm electrodes.

Implantable loop recorders in patients with atrial fibrillation.

INTRODUCTION: Implantable loop recorders (ILRs) provide practitioners with high-quality electrocardiographic data over an extended monitoring period. These data can guide the diagnosis and management of patients with atrial fibrillation (AF). AREAS COVERED: This review summarizes the available evidence and consensus statements supporting the use of ILRs in the detection of AF, as well as monitoring of patients with known AF. Future directions for research are also discussed. EXPERT OPINION: ILRs are the gold standard for detecting AF, providing superior diagnostic yield compared to other modes of ambulatory electrocardiography monitoring. Both experimental evidence and consensus statements support the use of ILRs in clinical settings where the diagnosis of AF may significantly change management, or where a high degree of sensitivity is needed. ILRs may also be used to monitor patients following AF ablation. More evidence is needed to better inform how ILR-detected AF should change management.

CACHET-CADB: A Contextualized Ambulatory Electrocardiography Arrhythmia Dataset.

ECG is a non-invasive tool for arrhythmia detection. In recent years, wearable ECG-based ambulatory arrhythmia monitoring has gained increasing attention. However, arrhythmia detection algorithms trained on existing public arrhythmia databases show higher FPR when applied to such ambulatory ECG recordings. It is primarily because the existing public databases are relatively clean as they are recorded using clinical-grade ECG devices in controlled clinical environments. They may not represent the signal quality and artifacts present in ambulatory patient-operated ECG. To help build and evaluate arrhythmia detection algorithms that can work on wearable ECG from free-living conditions, we present the design and development of the CACHET-CADB, a multi-site contextualized ECG database from free-living conditions. The CACHET-CADB is subpart of the REAFEL study, which aims at reaching the frail elderly patient to optimize the diagnosis of atrial fibrillation. In contrast to the existing databases, along with the ECG, CACHET-CADB also provides the continuous recording of patients' contextual data such as activities, body positions, movement accelerations, symptoms, stress level, and sleep quality. These contextual data can aid in improving the machine/deep learning-based automated arrhythmia detection algorithms on patient-operated wearable ECG. Currently, CACHET-CADB has 259 days of contextualized ECG recordings from 24 patients and 1,602 manually annotated 10 s heart-rhythm samples. The length of the ECG records in the CACHET-CADB varies from 24 h to 3 weeks. The patient's ambulatory context information (activities, movement acceleration, body position, etc.) is extracted for every 10 s interval cumulatively. From the analysis, nearly 11% of the ECG data in the database is found to be noisy. A software toolkit for the use of the CACHET-CADB is also provided.

Systemic sclerosis and tachycardia-bradycardia syndrome: a case report.

BACKGROUND: Systemic sclerosis is a multisystemic character autoimmune disease. It is characterized by vascular dysfunction and progressive fibrosis affecting mainly the skin but also different internal organs. All heart structures are commonly affected, including the pericardium, myocardium, and conduction system. However, tachycardia-bradycardia syndrome is not common in the literature as a cardiac complication of systemic sclerosis. Case presentation We report a case of tachycardia-bradycardia syndrome in a 46-year-old Moroccan woman followed for systemic sclerosis with cutaneous, vascular, and articular manifestations. The diagnosis was based mainly on patient-reported symptoms and electrocardiogram data. A permanent pacemaker was implanted, allowing the introduction of beta-blockers with good outcomes. CONCLUSIONS: This case aims to show that even minor electrocardiogram abnormalities should be monitored in this group of patients, preferably by 24-hour ambulatory electrocardiogram because they could be a good indicator of the activity and progression of cardiac fibrosis.

Improving stroke pathways using an adhesive ambulatory ECG patch: reducing time for patients to ECGs and subsequent results.

Three south-London hospital trusts undertook a feasibility study, comparing data from 93 patients who received the 14-day adhesive ambulatory electrocardiography (ECG) patch Zio XT with retrospective data from 125 patients referred for 24-hour Holter for cryptogenic stroke and transient ischaemic attack following negative 12-lead ECG. As the ECG patch was fitted the same day as the clinical decision for ambulatory ECG monitoring was made, median time to the patient having the monitor fitted was significantly reduced in all three hospital trusts compared with 24-hour Holter being ordered and fitted. Hospital visits reduced by a median of two for patients receiving Zio XT. This project supports that it is feasible to use a patch as part of routine clinical care with a positive impact on care pathways.

Skin sympathetic nerve activity in patients with chronic orthostatic intolerance.

BACKGROUND: Chronic orthostatic intolerance (OI) is characterized by the development of tachycardia and other symptoms when assuming an upright body position. OBJECTIVE: The purpose of this study was to test the hypothesis that skin sympathetic nerve activity (SKNA) bursts are specific symptomatic biomarkers in patients with chronic OI. METHODS: We used an electrocardiogram monitor with a built-in triaxial accelerometer to simultaneously record SKNA and posture in ambulatory participants. Study 1 compared chronic OI (14 women and 2 men; mean age 35 ± 10 years) with reference control participants (14 women; mean age 31 ± 6 years). Study 2 included 17 participants with chronic OI (15 women and 2 men; mean age 39 ± 12 years) not yet treated with ivabradine, pyridostigmine, or ß-blockers. RESULTS: In study 1, there were 124 episodes (8 ± 4 per participant) of postural changes, with 11 episodes (8.9%) associated with symptoms. In comparison, 0 of 104 postural changes (7 ± 3 per participant) in controls were symptomatic (P = .0011). In participants with chronic OI, the SKNA bursts associated with symptoms had higher burst frequencies, longer burst durations, and larger mean burst areas than did bursts during asymptomatic periods. However, SKNA bursts and tachycardia were asymptomatic in controls. We analyzed 110 symptomatic episodes in study 2 (6 ± 5 per participant). Among them, 98 (89.1%) followed at least 1 SKNA burst. In comparison, only 41 (37.3%) had heart rate exceed 100 beats/min 1 minute before symptom onset (P < .0001). CONCLUSION: SKNA bursts are a highly specific, albeit insensitive, symptomatic biomarker for chronic OI.

A cost comparison of atrial fibrillation monitoring strategies after embolic stroke of undetermined source.

Background: Detection of atrial fibrillation (AF) in patients with embolic stroke of undetermined source (ESUS) is challenging due to its paroxysmal nature. We sought to assess AF detection with an insertable cardiac monitor (ICM) and to perform cost analysis for various AF monitoring strategies post-ESUS We applied this cost analysis modeling to recently published Stroke AF and Per Diem trials. Methods: Retrospective chart review was performed in consecutive hospitalized patients with ESUS who had ICM placed prior to discharge. Utilizing rate of ICM-detected AF and Medicare average payments, we modeled 30-day per-patient diagnostic costs of Immediate ICM insertion prior to discharge versus using a wearable monitor followed by ICM in patients with ESUS, from Medicare and patient out-of-pocket perspectives. Similar modeling strategy and cost analysis was applied to the Stroke AF and Per Diem trials. Results: In 192 ESUS patients, AF detection increased with length of monitoring: 7.3 % at 14 days, 9.4 % at 30 days, and 17.2 % after a median ~ 6 months (189 days). Cost modeling predicted that immediate ICM leads to $3683-$4070 lower Medicare payments per-patient and $1425-$1503 lower patient out-of-pocket costs compared to Wearable-to-ICM strategies. Using similar modeling in the PER DIEM and STROKE AF trials, the additive costs of the 30-day ELR to ICM strategy ranged from $3786-$3946 from a payer perspective and $1472-$1503 from a patient out-of-pocket perspective. Conclusions: Use of ICM immediately after ESUS is cost-saving compared to Wearable-to-ICM strategies, due to the cost and low diagnostic yield of short-term wearable cardiac monitoring.

Ambulatory Electrocardiographic Monitoring Following Minimalist Transcatheter Aortic Valve Replacement.

OBJECTIVES: The aim of this study was to determine the impact of delayed high-degree atrioventricular block (HAVB) or complete heart block (CHB) after transcatheter aortic valve replacement (TAVR) using a minimalist approach followed by ambulatory electrocardiographic (AECG) monitoring. BACKGROUND: Little is known regarding the clinical impact of HAVB or CHB in the early period after discharge following TAVR. METHODS: A prospective, multicenter study was conducted, including 459 consecutive TAVR patients without permanent pacemaker who underwent continuous AECG monitoring for 14 days (median length of hospital stay 2 days; IQR: 1-3 days), using 2 devices (CardioSTAT and Zio AT). The primary endpoint was the occurrence of HAVB or CHB. Patients were divided into 3 groups: 1) no right bundle branch block (RBBB) and no electrocardiographic (ECG) changes; 2) baseline RBBB with no further changes; and 3) new-onset ECG conduction disturbances. RESULTS: Delayed HAVB or CHB episodes occurred in 21 patients (4.6%) (median 5 days postprocedure; IQR: 4-6 days), leading to PPM in 17 (81.0%). HAVB or CHB events were rare in group 1 (7 of 315 [2.2%]), and the incidence increased in group 2 (5 of 38 [13.2%]; P < 0.001 vs group 1) and group 3 (9 of 106 [8.5%]; P = 0.007 vs group 1; P = 0.523 vs group 2). No episodes of sudden or all-cause death occurred at 30-day follow-up. CONCLUSIONS: Systematic 2-week AECG monitoring following minimalist TAVR detected HAVB and CHB episodes in about 5% of cases, with no mortality at 1 month. Whereas HAVB or CHB was rare in patients without ECG changes post-TAVR, baseline RBBB and new-onset conduction disturbances determined an increased risk. These results would support tailored management using AECG monitoring and the possibility of longer hospitalization periods in patients at higher risk for delayed HAVB or CHB.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS).

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.

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.

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.