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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).

Published online by Cambridge University Press:  02 August 2021

Maully J. Shah*
University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania
Michael J. Silka*
University of Southern California Keck School of Medicine, Los Angeles, California
Jennifer N. Avari Silva
Washington University in St. Louis, St. Louis, Missouri
Seshadri Balaji
Oregon Health & Science University, Portland, Oregon
Cheyenne M. Beach
Yale University School of Medicine, New Haven, Connecticut
Monica N. Benjamin
Hospital de Pediatría Juan P. Garrahan, Hospital El Cruce, Hospital Británico de Buenos Aires, Instituto Cardiovascular ICBA, Buenos Aires, Argentina
Charles I. Berul
George Washington University, Washington, DC
Bryan Cannon
Mayo Clinic, Rochester, Minnesota
Frank Cecchin
New York University Grossman School of Medicine, New York, New York
Mitchell I. Cohen
Inova Children’s Hospital, Fairfax, Virginia
Aarti S. Dalal
Washington University in St. Louis, St. Louis, Missouri
Brynn E. Dechert
University of Michigan, Ann Arbor, Michigan
Anne Foster
Advocate Children’s Heart Institute, Chicago, Illinois
Roman Gebauer
Heart Centre Leipzig, University of Leipzig, Leipzig, Germany
M. Cecilia Gonzalez Corcia
Bristol Royal Hospital for Children, Bristol, UK
Prince J. Kannankeril
Vanderbilt University Medical Center, Nashville, Tennessee
Peter P. Karpawich
University Pediatricians, Children’s Hospital of Michigan, Detroit, Michigan
Jeffery J. Kim
Baylor College of Medicine, Houston, Texas
Mani Ram Krishna
Amrita Institute of Medical Sciences, Kochi, India
Peter Kubuš
Children’s Heart Center, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
Martin J. LaPage
University of Michigan, Ann Arbor, Michigan
Douglas Y. Mah
Harvard Medical School, Boston, Massachusetts
Lindsey Malloy-Walton
University of Missouri-Kansas City School of Medicine, Kansas City, Missouri
Aya Miyazaki
Shizuoka General Hospital and Mt. Fuji Shizuoka Children’s Hospital, Shizuoka, Japan
Kara S. Motonaga
Stanford University, Palo Alto, California
Mary C. Niu
University of Utah Health Sciences Center, Salt Lake City, Utah
Melissa Olen
Nicklaus Children’s Hospital, Miami, Florida
Thomas Paul
Georg-August-University Medical Center, Göttingen, Germany
Eric Rosenthal
Evelina London Children’s Hospital and St Thomas’ Hospital, Guy’s & St Thomas’ NHS Foundation Trust, London, UK
Elizabeth V. Saarel
St. Luke’s Health System, Boise, Idaho
Massimo Stefano Silvetti
Bambino Gesù Children’s Hospital IRCCS, Rome, Italy
Elizabeth A. Stephenson
The Hospital for Sick Children, Toronto, Canada
Reina B. Tan
New York University Langone Health, New York, New York
John Triedman
Harvard Medical School, Boston, Massachusetts
Nicholas H. Von Bergen
University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin
Philip L. Wackel
Mayo Clinic, Rochester, Minnesota
Author for correspondence: Dr Maully J. Shah, Cardiac Center, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104. E-mail:; OR Dr Michael J. Silka, Division of Pediatric Cardiology, Children’s Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, CA 90027. E-mail:
Author for correspondence: Dr Maully J. Shah, Cardiac Center, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104. E-mail:; OR Dr Michael J. Silka, Division of Pediatric Cardiology, Children’s Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, CA 90027. E-mail:
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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.

Original Article
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This is an open access article under the CC BY-NC-ND license (, which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is unaltered and is properly cited. The written permission of Cambridge University Press must be obtained for commercial re-use or in order to create a derivative work.
© 2021 The Author(s). Published by Elsevier Inc. on behalf of the Heart Rhythm Society and the American College of Cardiology Foundation, by Elsevier B.V. on behalf of the Indian Heart Rhythm Society, and by Cambridge University Press

Table of Contents


  1. Introduction

    1. Methodology and Evidence Review

    2. Organization of the Writing Committee

    3. Document Review and Approval

    4. Health Policy Objectives

    5. Top 10 Take-Home Messages

  2. Permanent Pacemakers

    1. Introduction

    2. Isolated Sinus Node Dysfunction

    3. Isolated Congenital Complete Atrioventricular Block

    4. Atrioventricular Block: Other Considerations

    5. Postoperative Atrioventricular Block

    6. Congenital Heart Disease: Specific Considerations

    7. Post Cardiac Transplantation

    8. Neuromuscular Diseases and Other Progressive Conduction Diseases

    9. Neurocardiogenic Syncope

    10. Cardiac Channelopathies

    11. Inflammation/Infection

  3. Implantable Cardioverter Defibrillators

    1. Introduction

    2. General Recommendations for ICD Therapy

    3. ICD Indications for Cardiac Channelopathies

      1. Long QT Syndrome

      2. Catecholaminergic Polymorphic Ventricular Tachycardia

      3. Brugada Syndrome

    4. ICD Indications for Cardiomyopathies

      1. Hypertrophic Cardiomyopathy

      2. Restrictive Cardiomyopathy

      3. Arrhythmogenic Cardiomyopathies

      4. Nonischemic Dilated Cardiomyopathies

    5. ICD indications for Congenital Heart Disease

  4. Insertable Cardiac Monitors

  5. CIED Lead Management

  6. CIED Follow-up and Ancillary Testing

  7. Special Considerations

    1. CIEDs and Magnetic Resonance Imaging

    2. CIEDs and Sports Participation

    3. CIEDs in Low- and Middle-Income Countries

    4. Shared Decision-Making

  8. Knowledge Gaps and Future Research

  9. References

  10. Appendix 1 Author Relationships With Industry

  11. Appendix 2 Reviewer Relationships With Industry

This article has been copublished in Heart Rhythm, JACC: Clinical Electrophysiology, Indian Pacing and Electrophysiology Journal, and Cardiology in the Young.


Guidelines for the implantation of cardiovascular implantable electronic devices (CIEDs) have evolved since the initial American College of Cardiology (ACC)/American Heart Association (AHA) pacemaker guidelines in 1984. Reference Frye, Collins and DeSanctis1 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 as well as other physiologic parameters. In view of the increasing complexity of both devices and patients in the current era, practice guidelines, by necessity, have become increasingly specific. One aspect of this evolution is the “2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay,” Reference Kusumoto, Schoenfeld and Barrett2 which included specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the need for differing indications for CIEDs as well as size-specific technology factors in younger patients. Therefore, this document has been developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, in recognition that there is often overlap in the care of patents between 18 and 21 years of age.

This document is an expert consensus statement intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This consensus statement will also provide guidance regarding the management of CIEDs for rhythm disorders in pediatric patients and address some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them.

Recommendations are presented in a modular or knowledge chunk format, in which each section includes a table of recommendations, a brief synopsis, and recommendation-specific supportive text. Reference Levine, O’Gara and Beckman3 However, this document is not intended to provide an exhaustive review of all aspects of pacemakers, ICDs, and insertable cardiac monitors (ICMs), as this information is easily accessible in electronic searches or textbooks. Furthermore, specific recommendations, such as heart rate criteria for pacemaker implantation, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. As a final introductory comment, to avoid clinical overlap, the indications and management of cardiac resynchronization therapy and physiological pacing will be addressed in the anticipated “2022 HRS Expert Consensus Statement on Cardiac Physiological Pacing for the Avoidance and Mitigation of Heart Failure,” which will include a specific section on pediatric and congenital heart disease (CHD).


Methodology and evidence review

The principles in the development of this document are 1) new recommendations and any changes to previous recommendations are based on data, when possible; 2) these recommendations are consistent with current ACC/AHA/Heart Rhythm Society (HRS) guidelines when reasonable; Reference Kusumoto, Schoenfeld and Barrett2Reference Indik, Gimbel and Abe19 and 3) all recommendations are critically reviewed, initially by the writing committee and editors, followed by the Pediatric and Congenital Electrophysiology Society (PACES) executive committee, and subsequently by external HRS, ACC, AHA, and Association for European Paediatric and Congenital Cardiology (AEPC) representatives. Any revisions or additions to existing recommendations will require approval of at least 80% by the members of the PACES writing committee. Specific prior guidelines and consensus statements relevant to CIEDs that have been referenced as the basis for recommendations in this document are acknowledged below and recognized in the specific sections (Table 1).

Table 1. Guidelines, expert consensus statements, and reports Cited

ECS = expert consensus statements; EHRA = European Heart Rhythm Association; ESC = European Society of Cardiology.

These recommendations have been developed consistent with standard guideline methodology, i.e., with both a class of recommendation (COR) and a level of evidence (LOE) (Table 2). Reference Halperin, Levine and Al-Khatib4 The class of the recommendation indicates the strength of recommendation, based on the estimated magnitude or certainty of benefit in proportion to risk. The level of evidence rates the quality of scientific evidence supporting the intervention on the basis of the type, quantity, and consistency of data from clinical trials and other sources. Due to the lack of randomized clinical trials (RCTs) in pediatric patients, these LOE recommendations will be limited to class B-NR (limited populations), class C-LD (very limited populations), or C-EO (consensus expert opinion, case studies, or standard of care). It is important to emphasize that a recommendation with a level of evidence C-EO does not imply that the recommendation is weak. Many of the questions addressed in this (and other) documents either do not lend themselves to clinical trials or are rare disease entities. Reference Kusumoto, Calkins and Boehmer5 However, there may be unequivocal expert consensus that a particular intervention is either effective or necessary. The final evidence tables for the recommendations are included in Supplemental Appendix 3 and summarize the evidence used by the writing committee to formulate these recommendations. References selected and published in this document are intended to be representative and not all-inclusive. Variations between previously published adult and pediatric CIED recommendations as well as new pediatric-specific recommendations are listed in Supplemental Appendix 4.

Table 2. Class of Recommendation and Level of Evidence Categories*

*Adapted from Halperin, et al.Reference Halperin, Levine and Al-Khatib4

Organization of the writing committee

The writing committee consisted of members of PACES who were selected by the PACES executive committee. The writing committee members included junior and senior pediatric electrophysiologists as well as allied health professionals and represented diverse genders, countries, and cultures. The writing committee also included external representatives from the ACC, AHA, HRS, and AEPC. Prior to final publication, all committee members were required to verify their specific contributions to this document. Appendix 1 lists writing committee members’ relevant relationships with industry.

Document review and approval

Following internal review by the PACES executive committee, this document was then reviewed by the PACES writing committee. Following considerations of these comments and approval by an independent PACES reviewer, the recommendations were opened for public comment to PACES members. An official reviewer each nominated by HRS, ACC, AHA, and AEPC provided independent external review. This document was then approved for publication by the PACES executive committee and endorsed by all collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society. Appendix 2 lists reviewers’ relevant relationships with industry.

Health policy objectives

The purpose of this document is to provide guidance to clinicians for the management of pediatric patients who may require a CIED, with a primary focus on the indications for device implantation. The document will be useful to pediatric cardiologists, cardiac surgeons, cardiac intensivists, anesthesiologists, and arrhythmia specialists. This document supersedes the pediatric CIED recommendations made in “ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities” Reference Epstein, Dimarco and Ellenbogen6 and “2012 ACCF/AHA/HRS Focused Update of the 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities.” Reference Tracy, Epstein and Darbar7 This document is expected to provide support for clinicians and patients to allow for appropriate device use, appropriate device management, follow-up, and appropriate reimbursement in pediatric patients.

Top 10 take-home messages

  1. 1. In patients with isolated sinus node dysfunction (SND), there is no minimum heart rate or maximum pause duration where permanent pacing is absolutely recommended. Establishing a temporal correlation between symptoms and bradycardia is critical in the decision as to whether permanent pacing is indicated.

  2. 2. Young patients with impaired ventricular function or abnormal cardiovascular physiology may be symptomatic due to sinus bradycardia or the loss of atrioventricular (AV) synchrony at heart rates that do not produce symptoms in individuals with normal cardiovascular physiology.

  3. 3. Although the average ventricular rate in newborns and infants with congenital complete atrioventricular block (CCAVB) provides an objective measure regarding the decision for pacemaker implantation, additional factors may equally influence the decision/timing of pacemaker implant. These include birth weight (size), congenital heart defects, ventricular function, and other comorbidities.

  4. 4. In patients with postoperative AV block, a period of observation for at least 7–10 days before pacemaker implantation remains advised; in select cases, earlier pacemaker implantation may be considered if AV block is not expected to resolve due to extensive injury to the cardiac conduction system.

  5. 5. Atrial pacing with antitachycardia pacing capabilities is reasonable for CHD patients with recurrent intra-atrial reentrant tachycardia when medication and catheter ablation are not effective.

  6. 6. There is increased recognition of the need for pacemaker implantation in conditions such as Kearns-Sayre syndrome or certain neuromuscular disorders due to the unpredictable progression of conduction disease.

  7. 7. The cause of sudden cardiac arrest (SCA) remains undefined in nearly 50% of pediatric survivors. ICD implantation is recommended provided completely reversible causes have been excluded, other treatments that may be beneficial are considered, and meaningful survival is anticipated.

  8. 8. The decisions for implantation of an ICD for primary prevention in cardiac channelopathies or cardiomyopathies remain guided by limited and, at times, conflicting data. Consideration of patient-specific factors and shared decision-making are critically important.

  9. 9. In pediatric patients with nonischemic dilated cardiomyopathy (NIDCM), primary prevention ICD implantation for left ventricular ejection fraction (LVEF) 35%, in the absence of other risk factors, is not clearly supported by published data.

  10. 10. In patients with indications for implantation of a CIED, shared decision-making and patient/family-centered care are endorsed and emphasized. Treatment decisions are based on the best available evidence and patient’s preferences.

Permanent pacemakers


The most common indications for permanent pacemaker implantation in children, adolescents, and patients with CHD may be classified as 1) symptomatic sinus bradycardia, 2) advanced second- or third-degree AV block, either congenital or acquired, and 3) pacing for the prevention or termination of tachyarrhythmias. Reference Epstein, Dimarco and Ellenbogen6 In general, many of the indications for pacemaker implantation in children and adolescents (defined as <19 years of age) are similar to those in adults. Reference Kusumoto, Schoenfeld and Barrett2 However, there are several important differences in infants and children. These patients have faster heart rates, and therefore standards for what is considered normal are age-dependent variables; whereas a heart rate of 45 bpm may be a normal in an adolescent, the same rate in a newborn or infant indicates profound bradycardia. In addition, young patients with impaired ventricular function or abnormal physiology may be symptomatic due to sinus bradycardia or loss of AV synchrony at heart rates that do not produce symptoms in individuals with normal cardiovascular physiology. Reference Hernández-Madrid, Paul and Abrams8,Reference Khairy, Van Hare and Balaji9 Hence, the indications for pacemaker implantation in young patients need to be based on the correlation of symptoms with relative bradycardia rather than absolute heart rate criteria.

Significant technical challenges may complicate device and lead implantation in small patients or those with abnormalities of venous or intracardiac anatomy. Epicardial pacemaker lead placement and use of device technology in innovative ways often need to be considered to provide pacing in the youngest patients. Reference Chang, Carter, Bar-Cohen, Shah, Rhodes and Kaltman20Reference Moore and Shannon22 Any pacemaker system used in a young patient may need to be utilized for multiple decades, and consideration of the long-term consequences from device and lead failure plays a role in implantation of pediatric devices.

Bradycardia and associated symptoms in children are often transient (e.g., breath-holding spells) and therefore may not require permanent pacemaker therapy. Conversely, there are other conduction system disorders that may rapidly progress (e.g., neuromuscular disorders) that may require prophylactic pacemaker implantation for disease-specific indications. In addition, as risk factors for cardiac conditions such as the channelopathies are better defined, the indications for device placement in these patients may evolve rapidly.

The goal of this section is to provide an update regarding the indications for permanent pacemaker implantation in pediatric patients. A summary of the recent literature is provided as a framework for clinicians to make individual decisions about pacing in these patients. As the pediatric and CHD populations represent unique groups of patients, clinical judgment and patient-specific decision-making are of the highest importance.

Isolated sinus node dysfunction

Recommendation-specific supportive text

SND refers to physiologically inappropriate atrial rates, due to either sustained bradycardia or abrupt pauses in the intrinsic cardiac rhythm. In patients with isolated sinus bradycardia without symptoms due to cerebral or systemic hypoperfusion, there is no minimum heart rate or maximum pause duration where permanent pacing is recommended. Establishing a temporal correlation between symptoms and age-related bradycardia is of paramount importance when determining whether permanent pacing is needed.

Nonrandomized studies in both children and adults have demonstrated that pacing can provide symptomatic improvement when symptoms, particularly syncope and pre-syncope, are clearly attributable to SND. Reference Breivik, Ohm and Segadal23Reference Chiu, Lin and Wang26 However, there is no clear evidence that pacing in the setting of isolated SND without symptoms improves outcomes.

In symptomatic patients with SND, atrial-based pacing is generally recommended over single-chamber ventricular pacing. Reference Kusumoto, Schoenfeld and Barrett2,Reference Kardelen, Celiker, Ozer, Ozme and Oto28 Furthermore, the decisions regarding pacemaker implantation for SND in patients with CHD or channelopathies should be made on an individualized basis and are discussed further in the corresponding sections. Reference Gillette, Wampler and Shannon29

Isolated congenital complete

Atrioventricular block

Recommendation-Specific Supportive Text

Although the average ventricular rate in newborns (≤30 days old) and infants (≤12 months old) with isolated CCAVB provides an objective measure regarding the decision for pacemaker implantation, additional factors may equally influence the decision/timing of pacemaker implant. These include birth weight (size), ventricular dysfunction, and other comorbidities. Reference Glatz, Rhodes and Gayno42 Furthermore, although symptoms such as poor feeding or tachypnea in the neonate may be due to multiple causes, they may be indicative of low cardiac output secondary to bradycardia. Therefore, a lower limit heart rate of 50 bpm is recommended for pacemaker implantation when overt symptoms related to low cardiac output do not appear to be present. One additional point of emphasis is that use of heart rate criteria for newborn or infant pacing should be based on heart rate consistency rather than a single measurement in time. Reference Michaëlsson and Engle34,Reference Pinsky, Gillette and Garson37

Beyond the first year of life, permanent pacemaker implantation is generally indicated in symptomatic patients. Contemporary studies suggest that approximately 66% of neonates and infants diagnosed with isolated CCAVB will undergo pacemaker implantation during their first year of life and that 90% of patients with CCAVB will undergo pacemaker implantation by 20 years of age. Reference Jaeggi, Hamilton and Silverman30 Long-term natural history studies have demonstrated progressive left ventricular dysfunction and mitral insufficiency with cardiovascular mortality in the fourth or fifth decade of life in patients with CCAVB who did not undergo pacemaker implantation. Reference Michaëlsson and Engle33,Reference Michaëlsson and Engle34,Reference Moak, Barron and Hougen43 On the other hand, some patients with CCAVB will develop left ventricular cardiomyopathy despite pacing due to either antibody-mediated myocarditis or pacing-induced dyssynchrony. Reference Moak, Barron and Hougen43,Reference Janoušek, van Geldorp and Krupičková44

Atrioventricular block: other considerations

Recommendation-specific supportive text

The diagnosis of advanced AV block during late childhood or adolescence is an uncommon but well-recognized phenomena. Advanced AV block may be congenital, may be related to infiltrative diseases, or may remain idiopathic. At times, late-onset AV block may be paroxysmal and quite difficult to document. Reference Silver, Pass and Hordof49

Exercise stress testing can be useful to detect the site and significance of AV block. Generally, supra-His block resolves with exercise by increased sympathetic tone. When second- and third-degree degree AV block are observed during exercise, conduction disturbance within the His-Purkinje system is suspected. Although progression to advanced second- and third-degree AV block during exercise is rare, it is associated with a poor prognosis in the absence of a pacemaker. Reference Yandrapalli, Harikrishnan, Ojo, Vuddanda and Jain47,Reference Bonikowske, Barout, Fortin-Gamero, Lara, Kapa and Allison48

With the exception of infiltrative or inflammatory causes of advanced AV block, the criteria for pacemaker implantation are similar to those for CCAVB. Permanent pacemaker implantation may be considered for advanced idiopathic AV block in adolescents with an acceptable ventricular rate, a narrow QRS complex, and normal ventricular function, based on an individualized consideration of symptoms and the risk/benefit ratio.

Postoperative atrioventricular block

Recommendation-specific supportive text

Postoperative AV block complicates 3–8% of congenital heart surgeries, with 1–3% of patients requiring permanent pacemaker implantation for persistent postoperative AV block. Reference Anderson, Czosek and Knilans56Reference Liberman, Pass and Hordof58 A very poor prognosis has been established for CHD patients with permanent postoperative AV block who do not receive permanent pacemakers. Reference Krongrad54,Reference Villain, Ouarda and Beyler55 Among patients who do regain AV conduction following a period of transient AV block, ≥85% have recovery of AV conduction by postoperative day 7 and ≥95% AV conduction by postoperative day 10. Reference Weindling, Saul and Gamble50,Reference Romer, Tabbutt and Etheridge51 Although patients who spontaneously regain AV conduction have a favorable prognosis, Reference Tracy, Epstein and Darbar7 there is a small but definite risk of late-onset complete AV block in transient postoperative AV block patients, with onset occurring as early as months, to as late as decades, following surgery. Reference Aziz, Serwer and Bradley52,Reference Krongrad54,Reference Villain, Ouarda and Beyler55 Limited data suggest that some patients with a history of transient postoperative advanced second- or third-degree AV block may be at risk for late-onset AV block or sudden cardiac death (SCD) if they have postoperative bifascicular block on the electrocardiogram (ECG) that was not present preoperatively. Reference Krongrad54,Reference Villain, Ouarda and Beyler55 Permanent pacemaker implantation may also be considered for transient postoperative third-degree AV block that reverts to intact AV node conduction when there is concern about the late development of AV block in patients with forms of CHD associated with progressive conduction abnormalities such as discordant AV connections, AV septal defects, and heterotaxy syndromes. Reference Huhta, Maloney and Ritter59,Reference Moore and Aboulhosn60

Congenital heart disease: specific considerations

Recommendation-specific supportive text

Patients with CHD often have important structural and functional lesions, Reference Stout, Daniels and Aboulhosn70 which influence both the indications for pacing as well as the type of pacing lead(s) utilized. Reference Glatz, Rhodes and Gayno42 Therefore, pacemaker implantation in these patients should not be viewed as an isolated procedure. The loss of vascular access or direct access to cardiac chambers and/or persistent right-to-left shunting require utilization of epicardial pacing leads (with concomitant sternotomy or thoracotomy), Reference Lau, Gaynor and Fuller74 although novel hybrid approaches to lead placement are being developed. Reference Termosesov, Kulbachinskaya and Polyakava75,Reference Clark, Kumthekar and Mass76

Bradycardia and scar-related tachycardias are common following surgery, and in the absence of high-grade AV block, atrial pacing is preferred to avoid pacing-induced ventricular dysfunction. Reference Tsao, Deal and Backer67,Reference Barber, Batra and Burch68 Permanent pacemaker and/or lead implantation may be considered prophylactically in patients with evidence of conduction disease and heart defects with a known natural progression to advanced heart block (e.g., discordant AV connections, heterotaxy syndrome) at the time of cardiac surgery. Reference Huhta, Maloney and Ritter59,Reference Moore and Aboulhosn60,Reference Cohen, Rhodes and Spray77

Similarly, in single-ventricle patients undergoing Fontan conversion, prophylactic antitachycardia pacemakers have been used. Reference Tsao, Deal and Backer67 There may be a role for pacing in improving the hemodynamic status in patients with plastic bronchitis and protein-losing enteropathy without conventional pacing indications. Reference Rychik, Atz, Celermajer and Deal78

The decisions regarding pacemaker implantation should also consider the complexity of the patient’s anatomy and hemodynamic status, with complex defined as patients with palliative repairs or impaired ventricular function or circulatory physiology. Reference Stout, Daniels and Aboulhosn70

Post cardiac transplantation

Recommendation-specific supportive text

Transient sinus bradycardia is relatively common immediately after transplantation and frequently resolves spontaneously. In rare cases, sinus bradycardia may persist and pacemaker implantation may be needed, but at least a week should be allowed for spontaneous recovery of sinus node function. Early post-transplant AV block has been reported in pediatric patients to be more frequent than in the adult population and may be related to donor age. Reference Kertesz, Towbin and Clunie79,Reference El-Assaad, Al-Kindi and Oliveira80 An analysis of the United Network for Organ Sharing (UNOS) database reported that between 1994 and 2014, 1% of cardiac transplant patients <18 years of age required a pacemaker in the acute post-transplant interval. Factors associated with need for pacemaker implant were biatrial anastomosis, older donor age, and antiarrhythmic drug use. Reference El-Assaad, Al-Kindi and Oliveira80

Late-onset conduction disorders (sinus node or AV node dysfunction) may be related to cardiac allograft vasculopathy or allograft rejection. Patients should be evaluated for the presence or development of transplant coronary artery disease, as late-onset bradycardia may be the first manifestation. Reference Kertesz, Towbin and Clunie79,Reference Cannon, Denfeld and Friedman84 Microvascular angiopathy that may not be seen during conventional angiography may also cause significant ventricular dysfunction and subsequent graft failure with an added risk for conduction abnormalities. Reference Chang, Hruban and Levin85

The role of prophylactic ICD implantation is not well established but may be considered in patients who require pacemakers. Risk factors to consider are coronary artery vasculopathy and left ventricular dysfunction, which may present as ventricular arrhythmias and have been associated with SCD. Reference Daly, Chakravarti and Tresler86,Reference Carboni87

Conditions include Duchenne muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy type 1, Friedreich ataxia, Emery-Dreifuss muscular dystrophy, facioscapulohumeral muscular dystrophy, Barth syndrome, Kearns-Sayre syndrome, lamin A/C mutations, and desmin-related myopathies.

Neuromuscular diseases and other

Progressive cardiac conduction diseases

Recommendation-specific supportive text

Progressive cardiac conduction diseases often involve genetic disorders with progressive deterioration of the conduction system occurring either in isolation or in conjunction with other cardiac and metabolic diseases including neuromuscular and mitochondrial diseases.

The severity and onset of cardiac complications differ among the diseases. Conduction disturbances are commonly observed in myotonic dystrophy type 1 and Emery-Dreifuss muscular dystrophy. Reference Jones, Mortsell and Rajaruthnam81 Variable degrees of conduction abnormalities may occur, ranging from first-degree AV block to complete AV block with unpredictable progression. Laminopathy caused by mutations in the LMNA gene is a wide-spectrum disorder exhibiting peripheral neuropathy, skeletal muscle disorders, progerias, and dilated cardiomyopathy. Cardiac conduction abnormalities, such as sinus bradycardia, AV block, atrial fibrillation, atrial standstill, and ventricular tachycardia (VT), are common and are often observed before the onset of heart failure symptoms. Reference Carboni87,Reference Groh, Groh and Saha92 In a meta-analysis, arrhythmias were observed in 36% of patients before 20 years of age, with heart failure observed in 10% before 30 years of age. Reference Carboni87 A prolonged PR interval >240 ms in adults is reported to be a predictor of progressive AV block and/or ventricular arrhythmias in patients with myotonic dystrophy and in patients with laminopathy. Reference Ha, Tarnopolsky and Bergstra91,Reference Groh, Groh and Saha92,Reference Van Berlo, de Voogt and van der Kooi94,Reference Hasselberg, Edvardsen, Petri and Berge99

Among the mitochondrial diseases, patients with Kearns-Sayre syndrome, characterized by progressive external ophthalmoplegia and myopathy with an onset before the age of 20 years, are known to carry a high risk for AV block and SCD. Reference Feingold, Mahle and Auerbach88Reference Ha, Tarnopolsky and Bergstra91 Currently, an HRS expert consensus statement on the evaluation and management of arrhythmic risk in neuromuscular disorders is under development. Therefore, the above recommendations may be subject to modification as newer data become available.

Neurocardiogenic syncope

Recommendation-specific supportive text

In the vast majority of cases, neurocardiogenic syncope is a limited disease and pacemaker implantation is not required. In some patients, however, recurrent syncopal events may significantly impair quality of life and may result in traumatic injury, particularly when the dominant feature of reflex syncope is cardioinhibitory. Reference Kolterer, Gebauer, Janousek, Dähnert, Riede and Paech101Reference Brignole, Menozzi and Moya104,Reference Bestawros, Darbar, Arain, Abou-Khalil, Plummer, Dupont and Rah108 Therefore, in a highly select group of patients who fail more conservative treatment options, pacemaker therapy may be useful by preventing profound bradycardia or prolonged asystole. Because the efficacy of pacing depends on the clinical setting, a clear relationship between symptoms and bradycardia should be established prior to pacemaker implantation. Bradycardia or asystole should be observed during episodes of clinical syncope, ideally on more than one occasion. Reference Paech, Wagner, Mensch and Antonin Gebauer105 Event monitors and ICMs have been effective for documenting this relationship.

In pallid breath-holding spells, studies of predominantly infants and toddlers have demonstrated either complete resolution or a significant reduction in the number of syncopal events in 86% patients with pacing. Reference Kolterer, Gebauer, Janousek, Dähnert, Riede and Paech101,Reference McLeod, Wilson, Hewitt, Norrie and Stephenson102 Single-chamber pacing with hysteresis appears as effective as dual-chamber pacing with rate drop response for the prevention of syncope and seizures. Pacemaker settings may be optimized to prevent sustained bradycardia by programming a relatively fast pacing rate at the time of the vasovagal reflex to augment cardiac output.

Attributed to vagal storm in the setting of epilepsy, ictal-induced bradyarrhythmia or asystole can impair both cerebral perfusion and cortical function and contribute to transient loss of consciousness and injury. Reference Sutton, de Jong and Stewart106,Reference Benditt, van Dijk and Thijs107 While conventional antiepileptic medications and epilepsy surgery are the mainstay treatments for ictal-induced bradycardia, pacemaker implantation may be reasonable as an adjunct for reducing the severity of symptoms.

Cardiac channelopathies

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The utility of pacing as adjunctive therapy in the various channelopathies is not well defined. Most data are based on observational reports of pacing in the context of long QT syndrome (LQTS). In certain high-risk patients with LQTS, permanent pacemaker implantation may provide a benefit to decrease bradycardia-related or pause-related initiation of ventricular tachyarrhythmias or so-called short-long-short episodes. Reference Moss, Liu and Gottlieb109Reference Viskin, Fish and Zeltser111 In infants with prolonged QT-related functional 2:1 AV block, one observational study reported that pacing in combination with other therapies resulted in favorable outcomes with no mortality. Reference Aziz, Tanel and Zelster112 Additionally, in some patients with LQTS, atrial pacing faster than the intrinsic rate has been shown to shorten the QT interval and reduce the rate of recurrent syncopal events in high-risk LQTS patients. Reference Moss, Liu and Gottlieb109,Reference Kowlgi, Giudicessi and Brake114 When SND and/or AV block are present in the setting of a channelopathy or as the result of antiarrhythmic medications needed for treatment of a channelopathy, the indications for permanent pacing detailed in the respective section on SND and/or AV block apply. In the setting of atrial standstill secondary to a channelopathy or laminopathy, single-chamber atrial pacemaker placement alone is not recommended due to the high probability of atrial noncapture. Reference Bellmann, Roser and Muntean115,Reference Ishikawa, Tsuji and Makita116


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Systemic infections may cause myocardial inflammation or infiltration presenting with bradycardia or complete AV block. Known causes are Lyme disease (Borrelia burgdorferi), Chagas disease in individuals from Trypanosoma cruzi–endemic areas in Central and South America, and rarely from diphtheria myocarditis. Other etiologies include infectious mononucleosis (Epstein-Barr virus), bacterial endocarditis, viral myocarditis with perivalvular abscess, rheumatic fever, and sarcoidosis.

In symptomatic AV block associated with Lyme disease, approximately 40% of patients may require temporary pacing, although AV block is typically reversible with antibiotic therapy. Reference McAlister, Klementowicz and Andrews117,Reference Forrester and Mead118 Chronic Chagas disease can present with different degrees of conduction defects. Advanced heart block in Chagas is permanent, and pacemaker implantation is indicated. Reference Nunes, Beaton and Acquatella119,Reference Bocchi, Bestetti and Scanavacca120 An ICD should be considered in Chagas cardiomyopathy in the presence of significant left ventricular dysfunction or ventricular arrhythmias. Reference Bocchi, Bestetti and Scanavacca120 More recently, there have been reports of transient AV conduction abnormalities associated with the COVID-19–related multisystem inflammatory syndrome in children (MIS-C) with ventricular dysfunction. Reference Dionne, Mah and Son121 Medical-directed therapy for the underlying condition should be maximized (including antibiotic therapy, steroids, intravenous immunoglobulins), and if tolerated, a waiting period of up to several months is warranted prior to pacemaker implantation to provide sufficient opportunity for spontaneous recovery of AV conduction.

Recovery of AV conduction in patients with complete heart block due to acute myocarditis has been reported to occur in 67% of young patients within 7 days of the onset of AV block. Reference Batra, Epstein and Silka122 Late monitoring for possible recurrence of symptoms or unrecognized recurrences of AV block or other arrhythmias is advised in these patients.

Implantable cardioverter defibrillators


The process of CIED guideline development has evolved over the past few decades, with initial recommendations based on observational clinical experience and refined based on controlled clinical studies and advances in device technology. Although the development of pediatric CIED recommendations has been limited by the lack of RCTs and small patient numbers, pacemaker recommendations have been established based on clearly defined diagnoses and five decades of clinical experience. Conversely, pediatric recommendations for ICD implantation have been primarily based on adult data and, with some modifications, applied to younger patients. Adult ICD guidelines are based on a specific diagnosis as the defined cause or presumed risk factor for a sudden cardiac event, such as ischemia, cardiomyopathy, or genetic cardiovascular disease. Reference Epstein, Dimarco and Ellenbogen6,Reference Tracy, Epstein and Darbar7,Reference Al-Khatib, Stevenson and Ackerman12,Reference Priori, Blomström-Lundqvist and Mazzanti13 In contrast, recent studies of pediatric SCA survivors have continued to demonstrate that in approximately 50% of cases, the cause of the event remains undefined despite an extensive and systematic evaluation. Reference Cunningham, Roston and Franciosi123,Reference Rucinski, Winbo and Marcondes124 Furthermore, in young patients with diagnoses such as catecholaminergic polymorphic ventricular tachycardia (CPVT) or Brugada syndrome, SCA is often the presenting symptom of the disease. Reference Silka, Kobayashi and Hill125,Reference van der Werf, Lieve and Bos126 Therefore, while development of pediatric ICD recommendations based on specific cardiovascular diagnoses would be intuitively preferable, the following discussion of ICD indications will begin with general considerations for the young patient with an unexplained SCA, followed by a more nuanced series of recommendations for ICD implantation when a specific cause of SCA or defined risk factor has been identified. Furthermore, there remain extensive “gaps” in current ICD recommendations, irrespective of age, for many of the diseases associated with SCD in pediatrics. Reference Cohen, Etheridge, Shah, Rhodes and Kaltman127,Reference Minier, Probst and Berthome128 The recommendations that follow are largely based on limited clinical data or expert opinion and consensus and require the application of case-specific clinical judgment and a shared-decision approach.

General recommendations for implantable cardioverter defibrillator therapy

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ICD guidelines specific to pediatrics must consider the unique aspects of device implantation and follow-up in children as well as the pathogenesis of the disease, which may evolve over time. A pediatric cardiologist should be involved in the decision to implant an ICD in pediatric patients, and the procedure should be performed by a cardiologist or cardiothoracic surgeon with special training and/or experience in CIED implantation in the pediatric age-group. ICD implantation should be a shared decision between the patient, family, and physician considering specific pediatric characteristics including age, size of the patient, need for an epicardial device, religious/cultural beliefs, and patient quality of life. This includes the physical as well as the psychological impact of an ICD on the patient’s well-being. Reference Sears, Hazelton and St Amant137 In addition, all ICD recommendations are based on the premise that meaningful survival of >1 year is expected; meaningful survival means that a patient has a reasonable quality of life and functional status. Reference Shen, Sheldon and Benditt11 It is further recommended that the indications for an individual patient’s ICD be reconsidered at each reintervention with respect to current guidelines, especially after a period of nonuse, as discontinuation of device therapy may be considered in select cases. Reference Kini, Soufi and Deo138

ICD indications for cardiac channelopathies

Long QT syndrome

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Congenital LQTS refers to genetically heterogeneous disorders characterized by the phenotypes of QTc prolongation on the ECG and risk of potentially life-threatening cardiac arrhythmias. Both phenotypic and genotypic characteristics are used to guide risk stratification of patients with LQTS and consideration for ICD. Reference Mazzanti, Maragna and Vacanti153 Phenotypic risk factors include the onset of symptoms at age <10 years, prior SCA, or recurrent syncope. Reference Bos, Bos and Johnson143Reference Biton, Rosero and Moss146,Reference Mazzanti, Maragna and Vacanti153 Additional high risk factors include a QTc ≥ 550 ms regardless of genotype, QTc ≥ 500 ms with LQT1 genotype, females with LQT2 genotype, and males with LQT3 genotype. Reference Wedekind, Burde and Zumhagen141,Reference Giudicessi and Ackerman150

Patients with rare conditions such as the Jervell and Lange-Nielson syndrome, Timothy syndrome, or calmodulinopathies may be at highest risk for SCA or SCD. Reference Giudicessi and Ackerman150Reference Crotti, Spazzolini and Tester152 Infants presenting with bradycardia, functional 2:1 AV block, or cardiac arrest are also at significant risk. Reference Moore, Gallotti and Shannon155

Nonselective beta-blockers are considered first-line therapy and can significantly decrease subsequent cardiac events in patients, especially in those with KCNQ1 mutations. Reference Priori, Wilde and Horie14,Reference Vincent, Schwartz and Denjoy140 In addition, beta-blockers and cardiac sympathetic denervation without ICD may be appropriate alternatives in carefully selected patients. Reference Priori, Wilde and Horie14,Reference Schwartz, Priori and Cerrone142,Reference Bos, Bos and Johnson143

In highest-risk patients, observational studies support effectiveness of the ICD in preventing SCD, with consideration of left cardiac sympathetic denervation to reduce the frequency of ICD shocks. Reference Schwartz, Spazzolini and Priori139,Reference Schwartz, Priori and Cerrone142,Reference Bos, Bos and Johnson143 However, implantation of an ICD in asymptomatic low-risk patient with LQTS for a positive family history of LQTS-related SCD is not clearly supported by published data, and individual decision-making is important. Reference Priori, Wilde and Horie14

Catecholaminergic polymorphic ventricular tachycardia

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CPVT is characterized by exertion-related polymorphic or bidirectional VT and is associated with syncope and SCA. SCA/SCD is reported in 3–13% of CPVT patients. Reference Priori, Napolitano and Memmi158 High risk factors include male sex, previous history of cardiac arrest, multiple genetic variants, and younger age at diagnosis. Reference Priori, Napolitano and Memmi158,Reference Roston, Haji-Ghassemi and LaPage159 Continued complex ventricular ectopy on exercise testing despite optimal medical therapy is also associated with worse outcome. Reference Hayashi, Denjoy and Extramiana160 Studies evaluating CPVT patients with >2 genetic variants suggest that these patients may also be at higher risk for SCA. Reference Roston, Haji-Ghassemi and LaPage159

Treatment with nonselective beta-blockers is associated with a reduction in adverse cardiac events. Reference Priori, Blomström-Lundqvist and Mazzanti13,Reference Priori, Wilde and Horie14,Reference Priori, Napolitano and Memmi158 The addition of flecainide to refractory patients in addition to maximally tolerated beta-blocker may suppress ventricular ectopy by as much as 85%. Reference Kannankeril, Moore and Cerrone161

In general, ICD implantation should be reserved for CPVT patients with prior SCA or with arrhythmogenic syncope on combination medical therapy and/or cardiac sympathetic denervation. Reference Priori, Blomström-Lundqvist and Mazzanti13,Reference Priori, Wilde and Horie14,Reference van der Werf, Lieve and Bos126,Reference Miyake, Webster and Czosek157 Inappropriate shocks are reported in 20–30% of CPVT patients with ICDs. Reference Miyake, Webster and Czosek157,Reference Olde Nordkamp, Postema and Knops163,Reference Roses-Noguer, Jarman and Clague164 Device programming in patients with CPVT should be optimized to deliver therapy for ventricular fibrillation (VF) and to minimize inappropriate shocks and the risk of potentially fatal electrical storms. Reference Miyake, Webster and Czosek157,Reference Roses-Noguer, Jarman and Clague164

Cardiac sympathetic denervation is recommended in patients who continue to have syncope or significant arrhythmias despite optimal medical therapy, are intolerant of medical therapy, or experience recurrent ICD shocks. Reference De Ferrari, Dusi and Spazzolini162 In selected patients with aborted SCA as the initial presentation of CPVT, pharmacologic therapy and/or cardiac sympathetic denervation without ICD may be considered as a possible alternative. Reference Priori, Wilde and Horie14,Reference van der Werf, Lieve and Bos126

Brugada syndrome

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Brugada syndrome (BrS) is an inherited arrhythmogenic disorder characterized by a coved-type ST-segment elevation in the right precordial ECG leads and an increased risk of SCD. Reference Al-Khatib, Stevenson and Ackerman12Reference Priori, Wilde and Horie14,Reference Gonzalez Corcia, de Asmundis and Chierchia165 The phenotypic expression of the disease spans from patients who are completely asymptomatic to those who experience a lethal arrhythmia. Reference Gonzalez Corcia, de Asmundis and Chierchia165,Reference Gonzalez Corcia, Sieira and Sarkozy166 The syndrome presents typically in the fourth to fifth decade, but in rare cases may have an early onset during childhood. Reference Moore, Gallotti and Shannon155 Pediatric cases are rare but can express as a rapidly progressive form and lead to life-threatening arrhythmias. Reference Minier, Probst and Berthome128,Reference Gonzalez Corcia, Sieira and Sarkozy166Reference Michowitz, Milman and Andorin169

The placement of an ICD remains the only therapy with proven efficacy for the management of ventricular arrhythmias and prevention of SCD in patients with BrS. Reference Gonzalez Corcia and Sieira170 Adult recommendations for risk stratification including ventricular stimulation have been established but have not been validated in pediatrics. Reference Al-Khatib, Stevenson and Ackerman12Reference Priori, Wilde and Horie14 Findings associated with high risk of ventricular arrhythmias and SCD in children include, in order of relevance: the presence of symptoms (SCD or arrhythmogenic syncope), spontaneous coved-type ST elevation (type I pattern) ECG, atrial arrhythmias and/or SND, and conduction abnormalities (AV block or intraventricular conduction delay). Reference Gonzalez Corcia, de Asmundis and Chierchia165 Although attempts have been made to create a noninvasive risk stratification scoring system, Reference Gonzalez Corcia, Sieira and Pappaert167 such recommendations are based on small cohorts. Patients with a type I ECG pattern and a history of syncope or SCD have a class I indication for an ICD implantation. Reference Hayashi, Denjoy and Extramiana160 In this study, 9 of 35 (26%) BrS patients with an ICD implanted at age <20 years received an appropriate therapy during a median follow-up of 7.3 years. Reference Hayashi, Denjoy and Extramiana160 Conversely, implantation of an ICD is not indicated in asymptomatic patients in the absence of risk factors. Large multicentric studies are necessary to further characterize risk factors and support primary prevention indications for BrS in pediatric patients.

ICD indications for cardiomyopathies

Hypertrophic cardiomyopathy

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Hypertrophic cardiomyopathy (HCM) is a genetic cardiovascular condition manifested by pathologic left ventricular hypertrophy in the absence of loading conditions. The phenotypic expression of HCM is variable, resulting in a diverse clinical course and highly variable long-term prognosis. Estimates for SCD rates in childhood HCM vary widely, with recent epidemiologic studies that have reported rates of between 1 and 7.2% per year. Reference Maron, Rowin and Casey172,Reference Miron, Lafreniere-Roula and Steve Fan174 While ICDs have improved the outcomes for patients with HCM resuscitated from SCA, the accurate identification of risk factors for SCD to guide primary prevention ICD implantation remains a challenge, particularly given the potential progression of the disease process over time. Reference Maron, Rowin and Casey172Reference Norrish, Cantarutti and Pissaridou175 A multicenter pediatric HCM registry study reported the 5-year risk of SCA was 9%. Reference Miron, Lafreniere-Roula and Steve Fan174 Primary and secondary prevention ICDs were implanted in 18 and 4% of the cohort, respectively. Only 2.5% of the patients with a primary prevention ICD received an appropriate discharge at 5 years’ follow-up, highlighting the major gaps in knowledge for accurate prediction of SCD risk in pediatric HCM patients. Reference Miron, Lafreniere-Roula and Steve Fan174

Previously published clinical practice guidelines define high risk for SCD in HCM by the presence of ≥1 clinical risk factors based on primarily adult data. Reference Epstein, Dimarco and Ellenbogen6,Reference Tracy, Epstein and Darbar7,Reference Priori, Wilde and Horie14 Recent studies, however, suggest that the significance of the various risk factors may differ in children compared to adults. Reference Miron, Lafreniere-Roula and Steve Fan174Reference Norrish, Ding and Field177 Conventional risk factors include survival from an SCA, spontaneous sustained VT, unexplained syncope, nonsustained VT, family history of early HCM-related SCD, and massive left ventricular hypertrophy. Reference Priori, Wilde and Horie14,Reference Maron, Spirito and Ackerman173 While a left ventricular wall thickness ≥30 mm is considered a risk factor in adults, left ventricular hypertrophy is determined relative to age and body size and therefore should be converted to a z score when evaluating this as a risk factor in smaller children. Reference Miron, Lafreniere-Roula and Steve Fan174,Reference Norrish, Ding and Field177 A multicenter pediatric study showed that a left ventricular posterior wall thickness z score ≥5 was associated with VT/VF or SCA, while a meta-analysis of pediatric studies reported a maximum left ventricular wall thickness ≥30 mm or a z score ≥6 associated with an increased risk of SCD. Reference Norrish, Cantarutti and Pissaridou175,Reference Balaji, DiLorenzo and Fish176

Other secondary risk factors for SCD, such as late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (MRI), have been investigated, but the predictive value of LGE for SCD in children is still unclear. Reference Briasoulis, Mallikethi-Reddy and Palla179,Reference Prinz, Schwarz and Ilic180 The evolving role of genetic testing for specific “malignant” sarcomere mutations remains debated and requires further investigation before inclusion as specific risk factors for SCD in pediatric patients with HCM. Reference Vermeer, Clur and Blom181,Reference Maron, Maron and Semsarian183

Restrictive cardiomyopathy

There are limited data regarding the use of ICDs in patients with restrictive cardiomyopathy. Reference Muchtar, Blauwet and Gertz184,Reference Walsh, Grenier and Jeffries185 The underlying cause of the restrictive cardiomyopathy is most commonly due to abnormalities in the sarcomeric genes, resulting in overlap with the HCM phenotype as well as risk for both tachyarrhythmias and conduction block. Reference Walsh, Grenier and Jeffries185 Given the overlap with HCM, ICD recommendations for patients with restrictive cardiomyopathy are included under the HCM and general guidelines. However, these patients do require unique consideration as, in comparison to those with HCM, patients with purely restrictive cardiomyopathy may not display the typical risk factors such as thickening of the intraventricular septum but do appear to be at higher risk for SCD, SCA, and cardiac transplant. Reference Webber, Lipshultz and Sleeper186,Reference Wittekind, Ryan and Gao187 Given this, ICD implantation may be appropriate in patients with a restrictive cardiomyopathy who present with heart failure or unexplained syncope when transplant is not an immediate option. Reference Zangwill, Naftel and L’Ecuyer188

Arrhythmogenic cardiomyopathies

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Arrhythmogenic cardiomyopathy (ACM) encompasses a spectrum of disorders of the myocardium with the distinguishing feature of presentation with sustained arrhythmias. Reference Towbin, McKenna and Abrams16 It includes, but is not limited to, genetic disorders such as arrhythmogenic right/left ventricular cardiomyopathy, lamin A/C, filamin-C, phospholamban, and cardiac amyloidosis. Reference Towbin, McKenna and Abrams16 Under this definition, infectious processes such as myocarditis and Chagas disease and inflammatory disorders such as sarcoidosis may also be classified. Most of these entities are infrequent before puberty and often overlap with other cardiomyopathies in presentation, particularly dilated cardiomyopathy. Reference Towbin, McKenna and Abrams16

The diagnosis of ACM requires a high degree of suspicion. The initial evaluation should include clinical history, physical examination, detailed family history, 12-lead ECG, echocardiography, ambulatory electrocardiography monitoring, exercise testing, and cardiac MRI. Additional testing includes signal-averaged ECG and genetic testing. Reference Towbin, McKenna and Abrams16,Reference DeWitt, Chandler and Hylind189

The most frequent form of ACM in the pediatric age-group is arrhythmogenic right ventricular cardiomyopathy (ARVC). Reference DeWitt, Chandler and Hylind189 ARVC is characterized by predominant right ventricular involvement with fibro-fatty replacement of the myocardium resulting in conduction abnormalities and ventricular arrhythmias. Biventricular disease is associated with younger age of onset. Reference Mazzanti, Ng and Faragli190,Reference Te Riele, James and Sawant191 ARVC is either de novo or inherited in an autosomal dominant pattern involving variances in desmosomal genes or desmosome-associated proteins. Reference Towbin, McKenna and Abrams16,Reference Ortiz-Genga, Cuenca and Dal Ferro193 Syncope is reported in 16–40% of ARVC patients at the time of diagnosis, is frequently exercise related, and has been associated with high arrhythmic risk. Reference Towbin, McKenna and Abrams16,Reference Te Riele, James and Sawant191 In adult ARVC cohorts, risk factors for SCD include syncope presumed due to ventricular arrhythmia, sustained or nonsustained VT, and severe right ventricular and/or left ventricular systolic dysfunction. Reference Al-Khatib, Stevenson and Ackerman12,Reference Towbin, McKenna and Abrams16 Due to the relatively low prevalence of manifest ARVC in the young, there is a paucity of data regarding risk stratification for SCD in pediatric patients with ARVC.

Overall, SCD affects 2–15% of young patients with ACM. Reference DeWitt, Chandler and Hylind189,Reference Te Riele, James and Sawant191 Patients presenting with SCD and/or sustained ventricular arrhythmias have a class I indication for an ICD implantation. Reference Al-Khatib, Stevenson and Ackerman12,Reference Towbin, McKenna and Abrams16 The limited available data on risk stratification in the young hamper the indication for a primary prevention ICD in this population. However, ICD implantation is reasonable in patients with ACM with hemodynamically tolerated sustained VT, syncope presumed due to ventricular arrhythmia, or an LVEF ≤ 35%. Candidacy and timing of cardiac transplantation and whether a wearable external defibrillator is a reasonable alternative should be taken into consideration on an individual basis for those patients with advanced heart failure. Reference Kusumoto, Calkins and Boehmer5

Nonischemic dilated cardiomyopathy

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The incidence of SCD in pediatric patients with idiopathic/NIDCM is only 1–5%, which is significantly less than that in adult patients. Reference Bharucha, Lee and Daubeney195,Reference Pahl, Sleeper and Canter196 Although studies have shown some ICD survival benefit for secondary prevention in pediatric dilated cardiomyopathy, the low incidence of SCD has made it quite difficult to establish risk factors to guide recommendations for primary prevention ICD implantation. Reference Bharucha, Lee and Daubeney195 In contrast to some studies of adult patients with NIDCM and LVEF ≤ 35%, Reference Bardy, Lee and Mark198 there is no clear evidence that ICDs implanted for primary prevention improve survival for pediatric patients with NICDM. Reference El-Assaad, Al-Kindi and Oliveira199,Reference Rhee, Canter and Basile200 However, primary prevention ICDs may be considered for patients with syncope or severe impairment of left ventricular function despite optimal medical therapy (beta-blockers and afterload reduction) and after careful consideration of device-related complication risks, candidacy and timing of cardiac transplantation, and whether a wearable external defibrillator is a reasonable alternative. Reference Kusumoto, Calkins and Boehmer5,Reference Kirk, Dipchand and Rosenthal17,Reference Dubin, Berul and Bevilacqua194,Reference Middlekauff, Stevenson, Stevenson and Saxon197

The phenotype of NIDCM may overlap with other types of pediatric cardiomyopathies resulting in variable risks of SCD. For example, the Sudden Death in Childhood Cardiomyopathy study showed that the risk of SCD varied according to cardiomyopathy phenotype. Reference Bharucha, Lee and Daubeney195 The cumulative incidence of SCD at 15 years was 5% for idiopathic dilated cardiomyopathy compared to 23% for left ventricular noncompaction. Myocardial dysfunction and/or a history of clinically significant arrhythmias are strongly associated with mortality in left ventricular noncompaction. Reference Jefferies, Wilkinson and Sleeper201,Reference Brescia202 Therefore, factors that may influence the decision regarding implantation of a primary prevention ICD include the underlying etiology of the NIDCM, the cardiomyopathy phenotype, the degree of ventricular dysfunction, and the presence of cardiac arrhythmias. Reference van Waning, Caliskan and Hoedemaekers203

ICD indications for congenital heart disease

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The association between CHD and arrhythmias has been well established. First demonstrated in repaired Tetralogy of Fallot, multiple studies since have identified risk factors for VT or SCD including residual cardiac defects, alterations in hemodynamics, and scars from prior interventions/surgeries. Reference Khairy, Harris and Landzberg204Reference Nieminen, Jokinen and Sairanen206 Correction of residual abnormalities or ablation of arrhythmogenic substrate may improve ventricular function and reduce symptoms. However, this may be inadequate to prevent the risk of subsequent VT or SCA in all but a select group of patients. Reference Miyazaki, Sakaguchi and Ohuchi207,Reference Zeppenfeld, Schalij and Bartelings208 ICD placement may therefore be appropriate in patients with, or at high risk of, potentially life-threatening arrhythmias. Reference Khairy, Van Hare and Balaji9,Reference Al-Khatib, Stevenson and Ackerman12,Reference Priori, Blomström-Lundqvist and Mazzanti13

While ICDs are commonly placed for both primary and secondary prevention in patients with CHD, those with CHD appear to have an increased risk of inappropriate shocks compared to those with ICDs and without CHD. Reference Berul, Van Hare and Kertesz130,Reference Von Bergen, Atkins and Dick131,Reference Jordan, Freedenberg and Wang211Reference Krause, Müller and Wilberg213 Appropriate ICD shock rates of 3–6% per year have been shown with an increased frequency of appropriate shocks for secondary prevention indications. Reference Khairy, Harris and Landzberg204 Antitachycardia pacing has been shown to be effective in VT termination and reducing ICD shocks. Reference Kalra, Radbill and Johns214 Patients with CHD receiving an ICD have an increased rate of complications as high as 26–45%, as well a high rate of inappropriate shocks. Reference Berul, Van Hare and Kertesz130,Reference Von Bergen, Atkins and Dick131,Reference Dechert, Bradley and Serwer212,Reference Krause, Müller and Wilberg213 The role of programmed stimulation and presence and degree of ventricular dysfunction as risk factors for SCD in CHD and thus primary prevention ICDs continues to be debated. Reference Sandhu, Ruckdeschel and Sauer215Reference Khairy, Landzberg and Gatzoulis217 ICD implantation can be especially challenging in patients with CHD due to anatomic complexity, intracardiac shunts, or limited vascular access. This may require nonstandard approaches such as epicardial leads, nontransvenous defibrillation coils or a subcutaneous ICD. Reference Radbill, Triedman and Berul218,Reference von Alvensleben Johannes, Dechert and Bradley David219

Insertable cardiac monitors

Syncope and palpitations are common symptoms in children and adolescents. ICMs (also referred to as implantable loop recorders) are subcutaneously implanted devices that provide long-term rhythm surveillance and documentation during a patient’s symptomatic event. Rhythm tracings during events are either patient-triggered recordings or stored automatically by predefined criteria. Long-term ECG monitoring using an ICM is recommended in symptomatic cases when the personal history, physical examination, and noninvasive investigations have been inconclusive, especially due to the low frequency of clinical events and/or limited feasibility of a complete diagnostic protocol. Reference Steinberg, Varma and Cygankiewicz220Reference Babikar, Hynes and Ward224 A remote monitoring program with immediate wireless data transfer capability and daily diagnostic data availability has overcome the prior problem of limited device storage capacity and has facilitated early diagnosis. ICMs, along with Holter monitoring, external loop recorders, and remote at-home telemetry, are reported to provide a diagnostic yield of 43–50% at 2 years and 80% at 4 years. Reference Moya, Sutton and Ammirati221Reference Placidi, Drago and Milioni226

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Several observational studies have demonstrated a benefit of ICM in establishing a diagnosis for recurrent symptoms of unclear etiology when other monitoring methods have failed to document an underlying cause.

CIED lead management

Lead management remains a vitally important issue in children, both with and without CHD. Updated consensus statements regarding lead management and extraction were put forth in 2017 Reference Kusumoto, Schoenfeld and Wilkoff18 and 2018. Reference Bongiorni, Burri and Deharo236 The following recommendations are complementary to these existing guidelines with a nuanced perspective focusing on pediatrics and patients with CHD.

The definitions used related to lead management in this document are similar to those explained in the 2017 statement. Reference Kusumoto, Schoenfeld and Wilkoff18 The general category of “lead removal” includes “lead explant” that is performed using a simple traction technique and “lead extraction” that refers to removal of a lead that has been implanted for >1 year or requiring the assistance of specialized equipment regardless of implant duration. Reference Kusumoto, Schoenfeld and Wilkoff18 The most common indications for transvenous lead extraction in children remain lead failure (76%) and venous occlusion. Reference Fu, Huang and Zhong237Reference McCanta, Kong and Carboni243 Pediatric patients are more likely to outlive the functionality of their leads, amplifying the importance of lead durability, longevity of venous access, and long-term risks of lead dysfunction. Coupled with studies in children indicating that older lead age is an independent predictor of need for advanced extraction techniques and added complexity, greater emphasis should be given to the potential risks of lead abandonment in this population. Reference Cecchin, Atallah and Walsh240,Reference Mah, Prakash and Porras241

Available extraction tools in children are similar to those in the adult population, as there are no special tools designed specifically for children or patients with CHD. These include locking stylets, telescoping sheaths, femoral snares, and mechanical, laser, or radiofrequency-powered sheaths. Reference McCanta, Kong and Carboni243,Reference Moak, Freedenberg and Ramwell244 Extractors should be appropriately trained, and the entire team must have working knowledge of these tools and techniques. Additionally, expertise in pediatrics, CHD, and surgically corrected anatomy is mandatory, as the methods and potential complications may be specific to both size and anatomy. Unusual lead position and foreign material such as prosthetic valves, conduits, and baffles may necessitate adjustments in approach. Reference Fender, Killu and Cannon242 Younger patients are also more likely to require the use of femoral extraction tools. Reference El-Chami, Sayegh and Patel245 Lastly, the presence of epicardial leads may require surgical access as a component of the procedure. Reference Mah, Prakash and Porras241

The environment for lead extractions in the pediatric population warrants careful patient-centered assessment for optimal preparedness (Table S1 in Supplemental Appendix 3). As in adults, major complications are relatively rare, but significant potential for life-threatening events exists. Reference Bongiorni, Burri and Deharo236,Reference Riley, Petersen, Ferguson and Bashir238,Reference Atallah, Erickson and Cecchin239 The contribution of complex CHD to the likelihood of successful extraction has varied, ranging from 74 to 94% for complete removal. The rates of major complications, however, have been found to be consistent between 3 and 4%. Reference Atallah, Erickson and Cecchin239,Reference Cecchin, Atallah and Walsh240,Reference Fender, Killu and Cannon242 Specific complications may be more prevalent based on anatomy and size, such as increased subpulmonary AV valve regurgitation in transposition of the great arteries, or increased risk of tricuspid or pulmonary valve involvement related to excess lead slack left for growth in smaller children. Reference McCanta, Kong and Carboni243Reference Webster, Margossian, Alexander, Cecchin, Triedman, Walsh and Berul248 Additionally, although patient age and size have not been shown to predict venous occlusion, more vigorous fibrous adhesions have been implicated in younger patients. Reference Bar-Cohen, Berul and Alexander249

Due to the complexities and potential for serious events in this population, lead extractions should only be performed in centers with an institutional commitment to the development and maintenance of a collaborative team. This includes a need for appropriate facilities, necessary equipment, trained personnel, and the ability to manage all complications. A multidisciplinary team familiar with nuances related to CHD is vital to maximizing procedural safety and efficacy (Table S1 in Supplemental Appendix 3). In particular, it is essential that the cardiac surgeon and surgical team be readily available to immediately provide open-chest surgical repair. Based on congenital anatomy and previous surgeries, emergent surgical approach via thoracotomy (versus sternotomy) may be preferred in certain scenarios, and focused pre-procedure imaging and planning is critical.

It must be recognized that several gaps in knowledge persist in relationship to lead management in children and patients with CHD. Reference Joy, Kumar and Poole250 This includes limited data in the very young, as well as the impact of multiple extractions over a lifetime on vascular integrity and valvular function. There also continues to be lack of clarity regarding prophylactic lead extractions at the time of generator change, Reference Sohal, Williams and Akhtar251 and long-term prospective studies on abandonment versus extraction in the young do not exist.

Recommendation-specific supportive text

The most common indications for lead removal are infection, venous occlusion, advisory or recall as a result of potential lead malfunction, or mechanical lead failure. Reference Kusumoto, Schoenfeld and Wilkoff18,Reference Joy, Kumar and Poole250Reference Escudero, Mah and Miyake256 Lead management involves the assessment of risks and benefits of whether or not to remove the lead based on the individual clinical condition of the patient as well as lead characteristics. Reference Kusumoto, Schoenfeld and Wilkoff18,Reference Bongiorni, Burri and Deharo236,Reference Viganego, O’Donoghue and Eldadah253

Upper extremity venous thrombosis and venous stenosis are not absolute indications for lead removal. However, instances in which a thrombosis causes significant symptoms (e.g., superior vena cava syndrome, ongoing thromboembolic events), or in which stenosis/occlusion impedes upgrade of an existing device, are generally considered appropriate circumstances to remove an existing lead. Reference Bongiorni, Burri and Deharo236Reference Riley, Petersen, Ferguson and Bashir238

Infections, which can result in CIED device and lead removal, can generally be grouped into major categories: isolated pocket infection, CIED-associated endocarditis, bacteremia without an alternative source (particularly Staphylococcus aureus), or bacteremia that persists or recurs despite appropriate antimicrobial therapy. Reference Kusumoto, Schoenfeld and Wilkoff18,Reference Bongiorni, Burri and Deharo236 These situations are associated with challenging management decisions and often require CIED device and lead removal when the infection is more than superficial cellulitis. Reference Viganego, O’Donoghue and Eldadah253,Reference Baddour, Epstein and Erickson254

Advisory/recall: The decision to remove an apparently normally functioning lead or leads in response to a manufacturer’s or regulatory body’s recall or warning is complex and should be performed in close consultation with an electrophysiologist with consideration for the patient’s overall clinical status. Reference Janson, Patel and Bonney255,Reference Escudero, Mah and Miyake256

Recommendations for CIED follow-up and ancillary testing

Recommendation-specific supportive text

Cardiovascular implantable electronic devices (CIEDs) that are currently amenable to remote interrogation and monitoring (RIM) include pacemakers, ICDs, and ICMs. The benefits of routine RIM are extensively validated and maximize the opportunity for prolongation of battery life as well as early detection and intervention of CIED malfunctions, arrhythmic issues, and adverse events. Reference Dechert, Sewer and Bradley268Reference Hummel, Leipold and Amorosi272 Remote evaluation of CIEDs began with transtelephonic monitoring (TTM), an analog-based technology that delivered limited data on pacemaker function via transmission over a telephone landline. RIM technologies, which are now incorporated in all CIEDs, are recommended over TTM because of the additional diagnostic data they provide, but TTM is still in use with older devices that do not have RIM capability. At present, there are no established guidelines for CIED follow-up in the pediatric population with resultant variability in monitoring of pediatric CIEDs. Reference Dechert, Sewer and Bradley273,Reference Boyer, Silka and Bar-Cohen274

Several device, lead, and pocket complications can be seen within the first few days to weeks after CIED implantation, and an in-person evaluation (IPE) is useful in the early post-implant phase. Although specific patient care guidelines for IPE and RIM for children have not been established, the Centers for Medicare & Medicaid Services has established reimbursement guidelines for IPE and RIM for patients with pacemakers.

In addition to monitoring the CIED itself, it is equally important to evaluate the impact of CIED-related consequences on the patient with ancillary testing. Ancillary testing may consist of but is not limited to 12-lead ECG, echocardiogram, ambulatory rhythm monitoring, chest X-ray, and exercise stress testing. The annual IPE should include evaluation of the patient’s underlying rhythm. In patients who have >40% paced ventricular rhythm, it is reasonable to assess systemic ventricular function by echocardiogram every 1–3 years for early recognition of pacemaker-induced cardiomyopathy or lead-related valve regurgitation. Reference Dasgupta, Madani and Figueroa259Reference Tantengco, Thomas and Karpawich263 Ambulatory rhythm monitoring and/or exercise stress testing may be useful in patients with arrhythmia concerns or symptoms related to activity and to assist with device optimization. Reference Gonzalez Corcia, Remy and Marchandise264Reference Diemberger, Gardini and Martignani267,Reference Kadish, Buxton and Kennedy275Reference Sampio, Craveiro and Darrieux277 It is reasonable to consider lead surveillance with chest X-ray in the acute post-implant period and to consider repeating every 1–3 years according to growth. Reference Mah, Prakash and Porras241,Reference Berul, Villafane and Atkins247

Special considerations

CIEDs and magnetic resonance imaging

Recommendation-specific supportive text

The 2017 MRI and Radiation Exposure in Patients with CIEDs Consensus Statement provides comprehensive recommendations for individuals with both conditional and nonconditional transvenous devices. Reference Indik, Gimbel and Abe19 With MRI, there is potential risk for heating of the lead, increase in pacing thresholds, sudden battery depletion, and inappropriate sensing/pacing. The consensus statement also provides guidance for CIED programming and evaluation pre-, during, and post-MRI along with a protocol of testing and patient-specific considerations. However, these recommendations are not specific for patients with abandoned or epicardial CIED leads and make no specific recommendations for MRI in these cases. Reference Nazarian, Hansford and Rahsepar283,Reference Padmanabhan, Kella and Mehta284

Regarding epicardial lead considerations, younger patients and those with CHD have a greater likelihood of requiring epicardial leads. Additionally, as there are no MRI conditional epicardial leads, even when used with a conditional device, the system is considered nonconditional. The 2017 recommendations suggest a possible contraindication to MRI, and in the pediatric section no recommendations regarding epicardial leads are made. However, when attached to a device, the limited data show only a small increase in risk for substantial alterations of the pacing threshold or changes in sensing after MRI. Reference Bireley, Kovach and Morton279Reference Gakenheimer-Smith, Etheridge and Niu281,Reference Rahsepar, Zimmerman and Hansford285,Reference Balmer, Gass and Dave286

Regarding abandoned leads, in vitro data suggest that epicardial leads are more likely to generate heat than transvenous leads; however, small studies evaluating MRIs in patients with both epicardial and transvenous abandoned leads suggest that it can be done safely in the majority of cases. Reference Schaller, Brunker and Riley282,Reference Nazarian, Hansford and Rahsepar283,Reference Langman, Goldberg and Finn287Reference Higgins, Gard and Sheldon289 Even so, these studies do not imply lack of an effect on the myocardium underlying the abandoned lead. In summary, the data on MRI use in epicardial or abandoned leads are inadequate to provide specific recommendations or an absolute contraindication.

Acknowledging the sparsity of data, but also appreciating the importance of MRI for diagnosis, prognosis, and surgical planning, individualized consideration of the risk/benefit ratio of MRI in young patients must be made on a “case-by-case basis.” Reference Indik, Gimbel and Abe19

CIEDs and sports participation

Recommendation-specific supportive text

The safety of sports participation for patients with CIEDs remained fundamentally unstudied until the past decade. Despite a dearth of research, initial published guidelines recommended against strenuous competitive sports participation (greater than class Ia) for patients with pacemakers or ICDs. Reference Zipes, Link and Ackerman295Reference Mitchell, Haskell and Snell298 Subsequent to publication of guidelines in 2005, evidence emerged suggesting that risks of sports participation for athletes with CIEDs may be lower than hypothesized. Reference Saarel, Pilcher and Etheridge290Reference Saarel, Law and Berul293

Surveys from HRS (2006) and PACES (2013) suggested that many patients with pacemakers and ICDs had participated in sports without adverse events. Reference Saarel, Pilcher and Etheridge290,Reference Mitchell, Haskell and Snell298 Thus, an international ICD Sports Registry was initiated and reported in 2013–2018. Reference Lampert, Olshansky and Heidbuchel292,Reference Saarel, Law and Berul293 The registry consisted of 129 patients <21 years of age including varsity high school and college athletes. While shocks occurred during sports, there were no deaths, no resuscitated arrests, and no arrhythmia-related injuries during sports. In addition, the rate of lead malfunction was similar to previously reported rates in unselected populations. Reference Lampert, Olshansky and Heidbuchel292 The conclusion was made that despite the potential for exercise to be arrhythmogenic, some young patients with ICDs can participate in sports without injury or failure to terminate the arrhythmia.

When questions arise about sports participation in youth with CIEDs, it is now standard practice to counsel patients and families about the risks, including potential for increased rate of ventricular tachyarrhythmias and damage to the pacemaker or ICD system. Counseling is patient specific; the underlying cardiac disease, type of device, indication for implant, position of leads and pulse generators, underlying heart rhythm, patient age, and type of athletic activity are considered when estimating risk. Reference Mitchell, Haskell and Snell298,Reference Lampert, Cannom and Olshansky299 Shared decision-making processes that include the patient, family, coach, school, team, and other community members should be utilized to determine the best course of pursuit for individuals with CIEDs and sporting endeavors.

CIEDs in low- and middle-income countries

A quote often used by doctors dealing with cardiac rhythm problems in resource limited settings (or indeed any branch of medicine) is the Italian proverb “Il meglio è l’inimico del bene,” which translates to “better is the enemy of good.” Low- and middle-income countries (LMIC) are defined as those designated by the World Bank based on per capita income. 300 They represent a heterogenous community including countries where the primary deterrent to the use of implantable devices is the cost of the device (India and most countries in Asia and Southern Africa) and those in which the deterrent is both the cost and the availability (sub-Saharan Africa). Reference Bonny, Mgantcha and Jeilan301 These problems have been alleviated to a small extent by philanthropic measures initiated by the Western world as well as universal health care policies announced by various governments in recent years. Pediatric cardiologists in these countries circumvent these problems by using two primary strategies:

  1. 1. Patient-specific strategy. Most centers in LMIC tailor the indications of the device to an individual patient instead of following standard guidelines. This is based on available evidence and is not anecdotal, as is widely believed. In postoperative heart block, it has been shown that 95% of AV conduction recovery happens by the 10th postoperative day. Reference Weindling, Saul and Gamble50,Reference Romer, Tabbutt and Etheridge51 Children with intermittent AV conduction on telemetry as well as an accelerated junctional rhythm have been shown to have a much higher recovery rate. Reference Murray, Smith and Flack302,Reference Paech, Dahnert and Kostelka303 Hence, many centers prefer to wait till the 10th postoperative day before placement of a permanent pacemaker. In children (and young adults) who have intermittent AV conduction and those with a reasonably fast narrow complex escape rhythm, centers may choose to wait even longer for recovery of AV conduction so as to avoid the use of a permanent pacemaker. Late recovery of surgically induced AV block has also been reported. Reference Paech, Dahnert and Kostelka303,Reference Batra, Wells and Hinoki304 Occasionally patients have been discharged home before return of AV conduction, and spontaneous recovery was documented on follow-up. Reference Bruckheimer, Berul and Kopf305 In patients with corrected CHD and normal ventricular function, a single-chamber pacemaker is used in most centers, while a dual-chamber pacemaker is reserved for children with palliated hearts and more than mild ventricular systolic dysfunction.

  2. 2. The use of explanted devices. Devices explanted from deceased patients with a battery life of >50% of a new device have been used in patients from a resource-limited setting. Reference Khairy, Lupien and Nava306 A hypothetical increased risk of infection from an explanted device has been a major deterrent for this approach. However, a recent meta-analysis of 18 studies involving 2270 patients in whom a reused pacemaker was placed revealed no significant increase in the risk of surgical site infection compared to a new device and a small increase in the risk of device malfunction. Reference Baman, Meier and Romero307 Even this small risk was shown to be predominantly technical and did not endanger the life of the patient. Standard guidelines on device reuse in India have been published. Reference Kapoor, Vora, Nataraj and Mishra308

While most centers have used such inventive strategies to implant a device in children, follow-up interrogation of the device is often challenging. Most pediatric cardiac centers in LMIC are located in a few urban centers with a very large referral area. Frequent travel for device interrogation is often impossible for families because of the costs involved as well as the loss of livelihood. There is no published literature on the gravity of this problem, as most centers lack the resources to follow patients meticulously. Although remote monitoring is ideally suited for these patients, the added cost of the device makes it less attractive. The recent launch of mobile-based remote monitoring pacemakers using Bluetooth technology has immense potential in LMIC if such devices can be priced affordably. Reference Roberts and El Refai309

Shared decision-making

Recommendation-specific supportive text

Shared decision-making is a process whereby patients, families, and providers exchange information and dialogue about medical diagnostic and treatment options. Reference Elwyn, Frosch and Thomson310 The goal is for patients and their families to reach evidence-informed and value-congruent medical decisions collaboratively with their clinicians. This modern model for health care decision-making has superseded paternalism, a previous model whereby providers made medical decisions on behalf of their patients using the ethical principal of beneficence. A shared decision-making approach, combining the ethical principles of professional beneficence and patient autonomy, has been shown to improve patient outcomes. Reference Greenfield and Kaplan311,Reference Legare, Adekpedjou and Stacey312

The use of shared decision-making should occur prior to all CIED implantation procedures. Clinicians must estimate and clearly describe the potential benefits and risks for the patient and their family. Some decisions will be relatively straightforward; for example, the decision to implant a permanent pacemaker to treat postoperative surgical complete heart block in a patient who is pacemaker dependent will be largely uncontestable. However, other treatment decisions, such as implantation of an ICD for primary prevention of SCD, are more complex and nuanced and include choice of ICD system, device location, and personalized estimation of risk of life-threatening arrhythmia for the particular patient over time.

Finally, the shared decision-making process is also important and applicable to post-implant diagnostic and treatment decisions for our patients with CIEDs including genetic testing, MRI, sports participation, pregnancy, cardiac surgery, and device reprogramming, removal, or revision.

Knowledge gaps and future research

There have been no RCTs involving CIEDs in children. Therefore, the recommendations put forth in this guideline are based on data from observational studies in children, clinical trials in adults, and expert opinion. Clinical trials, especially RCTs, remain challenging in pediatric populations because of low overall event rates in specific diseases and variations in disease progression from birth to adulthood. Reference Khairy, Dore and Poirier313

Critical knowledge gaps exist is several areas. Reference Goette, Auricchio and Boriani314 One example is the use of ICDs for the primary prevention of SCD. With reduction in size and the development of novel lead configurations, ICD use in pediatrics has increased dramatically while the age at implant has decreased significantly. Reference Berul, Van Hare and Kertesz130,Reference Burns, Evans and Kaltman315 However, the accurate identification of patients at increased risk remains perplexing.

Several other important knowledge gaps include but are not limited to the optimal timing of pacemaker implantation after postoperative AV block, contemporary outcomes of patients with isolated CCAVB who do not undergo pacing, risk factors for pacemaker-induced cardiomyopathy, optimal age and body size for transvenous lead implantation, and safety of MRI with abandoned or epicardial leads.

With continuing technological innovations, future research is needed to develop pediatric-specific criteria for application of these new technologies. These include subcutaneous ICDs, leadless pacemakers, and conduction system pacing. Reference von Alvensleben Johannes, Dechert and Bradley David219,Reference Breatnach, Dunne and Al-Alawi316,Reference Lyon, Dandamudi and Kean317 Multicenter prospective registries as well as high-quality retrospective data are necessary to provide real-world evidence for new and existing CIED technologies. Future research should be conducted in collaboration with PACES, other relevant scientific societies, the U.S. Food and Drug Administration, and industry partners for development of pediatric “appropriate” CIEDs and device algorithms to specifically benefit young patients and improve their long-term outcomes.

Supplementary data

Supplementary data (Appendices 3 and 4) associated with this article can be found in the online version at

Appendix A1

Author relationships with industry

Number value: 0 = $0; 1 = ≤$10,000; 2 = >$10,000 to ≤$25,000; 3 = >$25,000 to ≤$50,000; 4 = >$50,000 to ≤$100,000; 5 = >$100,000.

*Research and fellowship support are classed as programmatic support. Sources of programmatic support are disclosed but are not regarded as a relevant relationship with industry for writing group members or reviewers.

Appendix A2

Reviewer relationships with industry

Number value: 0 = $0; 1 = ≤$10,000; 2 = >$10,000 to ≤$25,000; 3 = >$25,000 to ≤$50,000; 4 = >$50,000 to ≤$100,000; 5 = >$100,000.

*Research and fellowship support are classed as programmatic support. Sources of programmatic support are disclosed but are not regarded as a relevant relationship with industry for writing group members or reviewers.


Representative of the Heart Rhythm Society (HRS)


Representative of the American College of Cardiology (ACC)


Representative of the Association for European Paediatric and Congenital Cardiology (AEPC)

Representative of the American Heart Association (AHA)


Maully J. Shah, MBBS, FHRS, FACC, and Michael J. Silka, MD, FACC, FAHA, are co-first authors.


Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1 for detailed information.



Frye, RL, Collins, JJ, DeSanctis, RW, et al. Guidelines for permanent pacemaker implantation, 1984. A report of the Joint American College of Cardiology/American Heart Association Task Force on Assessment of Cardiovascular Procedures (Subcommittee on Pacemaker Implantation). Circulation 1984; 70: 331A339A.Google Scholar
Kusumoto, FM, Schoenfeld, MH, Barrett, C, et al. 2018 ACC/AHA/HRS guideline on the evaluation and management of patients with bradycardia and cardiac conduction delay: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society. J Am Coll Cardiol 2019; 74: 932987.CrossRefGoogle Scholar
Levine, GN, O’Gara, PT, Beckman, JA, et al. Recent innovations, modifications, and evolution of ACC/AHA clinical practice guidelines: an update for our constituencies: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019; 139: e879e886.CrossRefGoogle Scholar
Halperin, JL, Levine, GN, Al-Khatib, SM, et al. Further evolution of the ACC/AHA clinical practice guideline recommendation classification system: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2016; 67: 15721574.CrossRefGoogle Scholar
Kusumoto, FM, Calkins, H, Boehmer, J, et al. HRS/ACC/AHA expert consensus statement on the use of implantable cardioverter-defibrillator therapy in patients who are not included or not well represented in clinical trials. Circulation 2014; 130: 94125.CrossRefGoogle ScholarPubMed
Epstein, AE, Dimarco, JP, Ellenbogen, KA, et al. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities. Heart Rhythm 2008; 5: 934955.CrossRefGoogle ScholarPubMed
Tracy, CM, Epstein, AE, Darbar, D, et al. 2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities. Heart Rhythm 2012; 9: 17371753.CrossRefGoogle ScholarPubMed
Hernández-Madrid, A, Paul, T, Abrams, D, et al. Arrhythmias in congenital heart disease. A position paper of the European Heart Rhythm Association, Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital Heart Disease. Europace 2018; 20: 17191753.CrossRefGoogle Scholar
Khairy, P, Van Hare, GF, Balaji, S, et al. 2014 PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease. Heart Rhythm 2014; 11: e102e165.CrossRefGoogle ScholarPubMed
Brignole, M, Auricchio, A, Baron-Esquivias, G, et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Europace 2013; 15: 10701118.Google Scholar
Shen, WK, Sheldon, RS, Benditt, DG, et al. 2017 ACC/AHA/HRS guidelines for the evaluation and management of patients with syncope. Circulation 2017; 136: e60e122.Google ScholarPubMed
Al-Khatib, SM, Stevenson, WG, Ackerman, MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. J Am Coll Cardiol 2018; 72: 16771749.CrossRefGoogle ScholarPubMed
Priori, SG, Blomström-Lundqvist, C, Mazzanti, A, et al. 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 2015; 36: 27932867.CrossRefGoogle ScholarPubMed
Priori, SG, Wilde, AA, Horie, M, et al. 2013 HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm 2013; 10: 19321963.CrossRefGoogle Scholar
Ommen, SR, Mital, S, Burke, MA, et al. 2020 AHA/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation 2020; 142: e558e631.Google Scholar
Towbin, JA, McKenna, WJ, Abrams, DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm 2019; 16: e301e372.CrossRefGoogle ScholarPubMed
Kirk, R, Dipchand, AI, Rosenthal, DN, et al. 2014 The International Society for Heart and Lung Transplantation guidelines for the management for pediatric heart failure. J Heart Lung Transplant 2014; 33: 888909.CrossRefGoogle ScholarPubMed
Kusumoto, FM, Schoenfeld, MH, Wilkoff, BL, et al. 2017 HRS expert consensus statement on cardiovascular implantable electronic device lead management and extraction. Heart Rhythm 2017; 14: e503e551.CrossRefGoogle ScholarPubMed
Indik, JH, Gimbel, JR, Abe, H, et al. 2017 HRS expert consensus statement on magnetic resonance imaging and radiation exposure in patients with cardiovascular implantable electronic devices. Heart Rhythm 2017; 14: e97e153.CrossRefGoogle ScholarPubMed
Chang, PM, Carter, C, Bar-Cohen, Y. Indications for permanent pacing, device and lead selection. In: Shah, M, Rhodes, L, Kaltman, J (eds). Cardiac Pacing and Defibrillation in Pediatric and Congenital Heart Disease. John Wiley and Sons Ltd, West Sussex, UK, 2017: 3761.CrossRefGoogle Scholar
Weindling, S, Saul, J, Triedman, J, et al. Staged pacing therapy for congenital complete heart block in premature infants. Am J Cardiol 1994; 74: 412413.CrossRefGoogle ScholarPubMed
Moore, JP, Shannon, KM. Transpulmonary atrial pacing: an approach to transvenous pacemaker implantation after extracardiac conduit Fontan surgery. J Cardiovasc Electrophysiol 2014; 25: 10281031.CrossRefGoogle ScholarPubMed
Breivik, K, Ohm, OJ, Segadal, L. Sick sinus syndrome treated with permanent pacemaker in 109 patients: a follow-up study. Acta Med Scand 1979; 206: 153159.CrossRefGoogle ScholarPubMed
Albin, G, Hayes, DL, Holmes, DR. Jr. Sinus node dysfunction in pediatric and young adult patients: treatment by implantation of a permanent pacemaker in 39 cases. Mayo Clin Proc 1985; 60: 667672.CrossRefGoogle Scholar
Gillette, PC, Shannon, C, Garson, A Jr., et al. Pacemaker treatment of sick sinus syndrome in children. J Am Coll Cardiol 1983; 1: 13251329.CrossRefGoogle ScholarPubMed
Chiu, SN, Lin, LY, Wang, JK, et al. Long-term outcomes of pediatric sinus bradycardia. J Pediatr 2013; 163: 885889.CrossRefGoogle Scholar
Reybrouck, T, Vangesselen, S, Gewillig, M. Impaired chronotropic response to exercise in children with repaired cyanotic congenital heart disease. Acta Cardiol 2009; 64: 723727.CrossRefGoogle ScholarPubMed
Kardelen, F, Celiker, A, Ozer, S, Ozme, S, Oto, A. Sinus node dysfunction in children and adolescent: treatment by placement of a permanent pacemaker in 26 patients. Turk J Pediatr 2002; 44: 312316.Google Scholar
Gillette, PC, Wampler, DR, Shannon, C, et al. Use of atrial pacing in a young population. Pacing Clin Electrophysiol 1985; 8: 94100.CrossRefGoogle Scholar
Jaeggi, ET, Hamilton, RM, Silverman, ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. J Am Coll Cardiol 2002; 39: 130137.CrossRefGoogle ScholarPubMed
Baruteau, AE, Fouchard, S, Behaghel, A, et al. Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study. Eur Heart J 2012; 33: 622629.CrossRefGoogle ScholarPubMed
Balmer, C, Fasnacht, M, Rahn, M, et al. Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy. Europace 2002; 4: 345349.CrossRefGoogle Scholar
Michaëlsson, M, Engle, MA. Isolated congenital complete atrioventricular block in adult life. Circulation 1995; 92: 442449.CrossRefGoogle ScholarPubMed
Michaëlsson, M, Engle, MA. Congenital complete heart block; an international study of the natural history. Cardiovasc Clin 1972; 4: 85101.Google Scholar
Winkler, RB, Freed, MD, Nadas, AS. Exercise induced ventricular ectopy in children and young adults with complete heart block. Am Heart J 1980; 9: 8792.CrossRefGoogle Scholar
Karpawich, PP, Gillette, PC, Garson, A Jr., et al. Congenital complete atrioventricular block: clinical and electrophysiologic predictors of need for pacemaker insertion. Am J Cardiol 1981; 48: 10981102.CrossRefGoogle ScholarPubMed
Pinsky, WW, Gillette, PC, Garson, A, et al. Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block. Pediatrics 1982; 69: 728733.Google ScholarPubMed
Dewey, RC, Capeless, MA, Levy, AM. Use of ambulatory electrocardiographic monitoring to identify high-risk patients with congenital complete heart block. N Engl J Med 1987; 316: 835839.CrossRefGoogle ScholarPubMed
Benson, DW, Spach, MS, Edwards, SB, et al. Heart block in children. Evaluation of subsidiary ventricular pacemaker recovery times and ECG tape recordings. Pediatr Cardiol 1982; 2: 3945.CrossRefGoogle ScholarPubMed
Sholler, GF, Walsh, EP. Congenital complete heart block in patients without anatomic cardiac defects. Am Heart J 1989; 118: 11931198.CrossRefGoogle ScholarPubMed
Kertesz, NJ, Friedman, RA, Colan, SD, et al. Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block. Circulation 1997; 96: 34303435.CrossRefGoogle ScholarPubMed
Glatz, AC, Rhodes, LA, Gayno, JW, et al. Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth. J Thorac Cardiovasc Surg 2008; 136: 767773.CrossRefGoogle ScholarPubMed
Moak, JP, Barron, KS, Hougen, TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol 2001; 37: 238242.CrossRefGoogle ScholarPubMed
Janoušek, van Geldorp, IE, Krupičková, S, et al. Permanent cardiac pacing in children: Choosing the optimal pacing site: a multicenter study. Circulation 2013; 127: 613623.CrossRefGoogle Scholar
Gladman, G, Davis, AM, Fogelman, R, Hamilton, RM, Gow, RM. Torsade de pointes, acquired complete heart block and inappropriately long QT in childhood. Can J Cardiol 1996; 12: 683685.Google ScholarPubMed
Strasberg, B, Kusniec, J, Erdman, S, et al. Polymorphous ventricular tachycardia and atrioventricular block. Pacing Clin Electrophysiol 1986; 9: 522526.CrossRefGoogle ScholarPubMed
Yandrapalli, S, Harikrishnan, P, Ojo, A, Vuddanda, VLK, Jain, D. Exercise induced complete atrioventricular block: utility of exercise stress test. J Electrocardiol 2018; 51: 153155.CrossRefGoogle Scholar
Bonikowske, AR, Barout, A, Fortin-Gamero, S, Lara, MIB, Kapa, S, Allison, TG. Frequency and characteristics of exercise-induced second-degree atrioventricular block in patients undergoing stress testing. J Electrocardiol 2019; 54: 5460.CrossRefGoogle ScholarPubMed
Silver, ES, Pass, RH, Hordof, A, Liberman. Paroxysmal AV block in children with normal cardiac anatomy as a cause of syncope. Pacing Clin Electrophysiol 2008; 31: 322326.CrossRefGoogle ScholarPubMed

Postoperative atrioventricular block

Weindling, SN, Saul, PJ, Gamble, WJ, et al. Duration of complete atrioventricular block after congenital heart disease surgery. Am J Cardiol 1998; 82: 525527.CrossRefGoogle ScholarPubMed
Romer, AJ, Tabbutt, S, Etheridge, SP, et al. Atrioventricular block after congenital heart surgery: analysis from the Pediatric Cardiac Critical Care Consortium. J Thorac Cardiovasc Surg 2019; 157: 11681177.CrossRefGoogle ScholarPubMed
Aziz, PF, Serwer, GA, Bradley, DJ, et al. Pattern of recovery for transient complete heart block after open heart surgery for congenital heart disease: duration alone predicts risk of late complete heart block. Pediatric Cardiol 2012; 34: 9991005.CrossRefGoogle ScholarPubMed
Gross, GJ, Chiu, CC, Hamilton, RM, et al. Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention. Heart Rhythm 2006; 3: 601604.CrossRefGoogle ScholarPubMed
Krongrad, E. Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects. Circulation 1978; 57: 867870.CrossRefGoogle ScholarPubMed
Villain, E, Ouarda, F, Beyler, C, et al. Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy. Arch Mal Coeur Vaiss 2003; 96: 495498.Google Scholar
Anderson, JB, Czosek, RJ, Knilans, TK, et al. Postoperative heart block in children with common forms of congenital heart disease: results from the KID Database. J Cardiovasc Electrophysiol 2012; 23: 13491354.CrossRefGoogle ScholarPubMed
Ayyildiz, P, Kasar, T, Ozturk, E, et al. Evaluation of permanent or transient complete heart block after open heart surgery for congenital heart disease. Pacing Clin Electrophysiol 2016; 9: 160165.CrossRefGoogle Scholar
Liberman, L, Pass, RH, Hordof, AJ, et al. Incidence and characteristics of heart block after heart surgery in pediatric patients: A multicenter study. J Thorac Cardiovasc Surg 2016; 152: 197202.CrossRefGoogle ScholarPubMed
Huhta, JC, Maloney, JD, Ritter, DG, et al. Complete atrioventricular block in patients with atrioventricular discordance. Circulation 1983; 67: 13741377.CrossRefGoogle ScholarPubMed
Moore, JP, Aboulhosn, JA. Introduction to the congenital heart defects: anatomy of the conduction system. Cardiac Electrophysiol Clin 2017; 9: 167175.CrossRefGoogle ScholarPubMed

Congenital heart disease: specific considerations

Jaeggi, ET, Hornberger, LK, Smallhorn, JF, et al. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol 2005; 26: 1621.CrossRefGoogle ScholarPubMed
Lopes, LM, Tavares, GM, Damiano, AP, et al. Perinatal outcome of fetal atrioventricular block: one-hundred-sixteen cases from a single institution. Circulation 2008; 118: 12681275.CrossRefGoogle ScholarPubMed
Silka, MJ, Manwill, JR, Kron, J, et al. Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates. Am J Cardiol 1990; 65: 488493.CrossRefGoogle Scholar
Rhodes, LA, Walsh, EP, Gamble, WJ, et al. Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease. Pacing Clin Electrophysiol 1995; 18: 10051016.CrossRefGoogle ScholarPubMed
Kramer, CC, Maldonado, JR, Olson, MD, et al. Safety and efficacy of atrial antitachycardia pacing in congenital heart disease. Heart Rhythm 2018; 15: 543547.CrossRefGoogle ScholarPubMed
Stephenson, EA, Casavant, D, Tuzi, J, et al. Efficacy of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease. Am J Cardiol 2003; 92: 871876.CrossRefGoogle ScholarPubMed
Tsao, S, Deal, BJ, Backer, CL, et al. Device management of arrhythmias after Fontan conversion. J Thorac Cardiovasc Surg 2009; 138: 937940.CrossRefGoogle ScholarPubMed
Barber, BJ, Batra, AS, Burch, GH, et al. Acute hemodynamic effects of pacing in patients with Fontan physiology: a prospective study. J Am Coll Cardiol 2005; 46: 19371942.CrossRefGoogle ScholarPubMed
Drago, F, Silvetti, MS, Grutter, G, et al. Use of DDDRP pacing device in prevention and treatment of tachy-brady syndrome after Mustard procedure. Pacing Clin Electrophysiol 2004; 27: 530532.CrossRefGoogle ScholarPubMed
Stout, KK, Daniels, CJ, Aboulhosn, JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2019; 139: e698e800.Google Scholar
Khairy, P, Landzberg, M, Gatzoulis, MA, et al. Epicardial versus ENdocardial pacing and Thromboembolic events (EVENT) Investigators. Transvenous pacing leads and systemic thromboemboli in patients with intracardiac shunts: A multicenter study. Circulation 2006; 113: 23912397.CrossRefGoogle ScholarPubMed
DeSimone, CV, Friedman, PA, Noheria, A, et al. Stroke or transient ischemic attack in patients with transvenous pacemaker or defibrillator and echocardiographically detected patent foramen ovale. Circulation 2013; 128: 14331441.CrossRefGoogle ScholarPubMed
Supple, GE, Ren, J-F, Zado, ES, Marchlinski, FE. Mobile thrombus on device leads in patients undergoing ablation. Circulation 2011; 124: 772778.CrossRefGoogle Scholar
Lau, KC, Gaynor, JW, Fuller, SM, et al. Long term atrial and ventricular epicardial pacemaker lead survival after cardiac operations in pediatric patients with congenital heart disease. Heart Rhythm 2015; 12: 566573.CrossRefGoogle ScholarPubMed
Termosesov, S, Kulbachinskaya, E, Polyakava, E, et al. Video-assisted thoracoscopic pacemaker lead placement in children with atrioventricular block. Ann Pediatr Cardiol 2021; 14: 6771.CrossRefGoogle Scholar
Clark, B, Kumthekar, R, Mass, P, et al. Chronic performance of subxiphoid minimally invasive pericardial Model 20066 pacemaker lead insertion in an infant animal model. J Interv Card Electrophysiol 2020; 59: 1319.CrossRefGoogle Scholar
Cohen, MI, Rhodes, LA, Spray, TL. Efficacy of prophylactic epicardial pacing leads in children and young adults. Ann Thorac Surg 2004; 78: 197203.CrossRefGoogle ScholarPubMed
Rychik, J, Atz, AM, Celermajer, DS, Deal, BJ, et al. Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association. Circulation 2019; 140: e234e284.CrossRefGoogle Scholar

Post cardiac transplantation

Kertesz, NJ, Towbin, JA, Clunie, S, et al. Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection. J Heart Lung Transplant 2003; 22: 889893.CrossRefGoogle Scholar
El-Assaad, I, Al-Kindi, SG, Oliveira, GH, et al. Pacemaker implantation in pediatric heart transplant recipients: predictors, outcomes, and impact on survival. Heart Rhythm 2015; 12: 17761781.CrossRefGoogle ScholarPubMed
Jones, DG, Mortsell, DH, Rajaruthnam, D, et al. Permanent pacemaker implantation early and late after heart transplantation: clinical indication, risk factors and prognostic implications. J Heart Lung Transplant 2011; 30: 12571265.CrossRefGoogle ScholarPubMed
Mahmood, A, Andrews, R, Fenton, M, et al. Permanent pacemaker implantation after pediatric heart transplantation: risk factors, indications, and outcomes. Clin Transplant 2019; 33: e13503.CrossRefGoogle ScholarPubMed
Luebbert, JJ, Lee, FA, Rosenfeld, LE. Pacemaker therapy for early and late sinus node dysfunction in orthotopic heart transplant recipients: a single-center experience. Pacing Clin Electrophysiol 2008; 31: 11081112.CrossRefGoogle ScholarPubMed
Cannon, BC, Denfeld, SW, Friedman, RA, et al. Late pacemaker requirement after pediatric orthotopic heart transplantation may predict the presence of transplant coronary artery disease. J Heart Lung Transplant 2004;23:6771.CrossRefGoogle ScholarPubMed
Chang, AC, Hruban, RH, Levin, HR, et al. Comparison of rejection in the atrioventricular node and bundles with the working myocardium in transplanted hearts. J Heart Lung Transplant 1991; 10: 915920.Google ScholarPubMed
Daly, KP, Chakravarti, SB, Tresler, M, et al. Sudden death after pediatric heart transplantation: analysis of data from the Pediatric Heart Transplant Study Group. J Heart Lung Transplant 2011; 30: 13951402.CrossRefGoogle ScholarPubMed
Carboni, MP. Sudden cardiac death after heart transplantation: can ICD prevent SCD? Heart Rhythm 2014; 11: 16911692.CrossRefGoogle Scholar

Neuromuscular diseases and other progressive conduction diseases

Feingold, B, Mahle, WT, Auerbach, S, et al. Management of cardiac involvement associated with neuromuscular diseases: A scientific statement from the American Heart Association. Circulation 2017; 136: e200e231.CrossRefGoogle ScholarPubMed
Bhakta, D, Shen, C, Kron, J, et al. Pacemaker and implantable cardioverter-defibrillator use in a us myotonic dystrophy type 1 population. J Cardiovasc Electrophysiol 2011; 22: 13691375.CrossRefGoogle Scholar
Lund, M, Diaz, KJ, Ranthe, MF, et al. Cardiac involvement in myotonic dystrophy: A nationwide cohort study. Eur Heart J 2014; 35: 21582164.CrossRefGoogle ScholarPubMed
Ha, AH, Tarnopolsky, MA, Bergstra, TG, et al. Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II. Pacing Clin Electrophysiol 2012; 35: 12621269.CrossRefGoogle ScholarPubMed
Groh, WJ, Groh, MR, Saha, C, et al. Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1. N Engl J Med 2008; 358: 26882697.CrossRefGoogle ScholarPubMed
Wahbi, K, Meune, C, Porcher, R, et al. Electrophysiological study with prophylactic pacing and survival in adults with myotonic dystrophy and conduction system disease. JAMA 2012; 307: 12921301.CrossRefGoogle ScholarPubMed
Van Berlo, JH, de Voogt, WG, van der Kooi, AJ, et al. Meta-analysis of clinical characteristics of 299 carriers of LMNA gene mutations: Do lamin A/C mutations portend a high risk of sudden death? J Mol Med 2005; 83: 7983.Google Scholar
Polak, PE, Zijlstra, F, Roelandt, JR. Indications for pacemaker implantation in the Kearns-Sayre syndrome. Eur Heart J 1989; 10: 281282.CrossRefGoogle ScholarPubMed
Kabunga, P, Lau, AK, Phan, K, et al. Systematic review of cardiac electrical disease in Kearns-Sayre syndrome and mitochondrial cytopathy. Int J Cardiol 2015; 181: 303310.CrossRefGoogle ScholarPubMed
Khambatta, S, Nguyen, DL, Beckman, TJ, et al. Kearns-Sayre syndrome: A case series of 35 adults and children. Int J Gen Med 2014; 7: 325332.Google ScholarPubMed
Di Mambro, C, Tamborrino, PP, Silvetti, MS, et al. Progressive involvement of cardiac conduction system in paediatric patients with Kearns-Sayre syndrome: how to predict occurrence of complete heart block and sudden cardiac death? Europace 2021; 6: 948957.CrossRefGoogle Scholar
Hasselberg, NE, Edvardsen, T, Petri, H, Berge, KE, et al. Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects. Europace 2014; 16: 563571.CrossRefGoogle ScholarPubMed
Asatryan, B, Medeiros-Domingo, A. Molecular and genetic insights into progressive cardiac conduction disease. Europace 2019; 21: 11451158.CrossRefGoogle ScholarPubMed

Neurocardiogenic syncope

Kolterer, B, Gebauer, RA, Janousek, J, Dähnert, I, Riede, FT, Paech, C. Improved quality of life after treatment of prolonged asystole during breath holding spells with a cardiac pacemaker. Ann Pediatr Cardiol 2015; 8: 113117.Google ScholarPubMed
McLeod, KA, Wilson, N, Hewitt, J, Norrie, J, Stephenson, JB. Cardiac pacing for severe childhood neurally mediated syncope with reflex anoxic seizures. Heart 1999; 82: 721725.CrossRefGoogle ScholarPubMed
Kelly, AM, Porter, CJ, McGoon, MD, Espinosa, RE, Osborn, MJ, Hayes, DL. Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation. Pediatrics 2001; 108: 698702.CrossRefGoogle Scholar
Brignole, M, Menozzi, C, Moya, A, et al. Pacemaker therapy in patients with neurally mediated syncope and documented asystole: third International Study on Syncope of Uncertain Etiology (ISSUE-3): a randomized trial. Circulation 2012; 125: 25662571.CrossRefGoogle Scholar
Paech, C, Wagner, F, Mensch, S, Antonin Gebauer, R. Cardiac pacing in cardioinhibitory syncope in children. Congenit Heart Dis 2018; 13: 10641068.CrossRefGoogle ScholarPubMed
Sutton, R, de Jong, JSY, Stewart, JM, et al. Pacing in vasovagal syncope: physiology, pacemaker sensors, and recent clinical trials-Precise patient selection and measurable benefit. Heart Rhythm 2020; 17: 821828.CrossRefGoogle ScholarPubMed
Benditt, DG, van Dijk, G, Thijs, RD. Ictal asystole: life-threatening vagal storm or a benign seizure self-termination mechanism? Circ Arrhythm Electrophysiol 2015; 8: 1114.CrossRefGoogle ScholarPubMed
Bestawros, M, Darbar, D, Arain, A, Abou-Khalil, B, Plummer, D, Dupont, WD, Rah, SR. Ictal Asystole and Ictal Syncope: insights into Clinical Management. Circ Arrhythm Electrophysiol 2015; 8: 159164.CrossRefGoogle ScholarPubMed

Cardiac channelopathies

Moss, AJ, Liu, JE, Gottlieb, S, et al. Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome. Circulation 1991; 84: 15241529.CrossRefGoogle ScholarPubMed
Eldar, M, Griffin, JC, Van Hare, GF, et al. Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome. J Am Coll Cardiol 1992; 20: 830837.CrossRefGoogle ScholarPubMed
Viskin, S, Fish, R, Zeltser, D, et al. Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent? Heart 2000; 83: 661666.CrossRefGoogle ScholarPubMed
Aziz, PF, Tanel, RE, Zelster, IJ, et al. Congenital long QT syndrome and 2:1 atrioventricular block: an optimistic outcome in the current era. Heart Rhythm 2010; 7: 781785.CrossRefGoogle ScholarPubMed
Eldar, M, Griffin, JC, Abbott, JA, et al. Permanent cardiac pacing in patients with the long QT syndrome. J Am Coll Cardiol 1987; 10: 600607.CrossRefGoogle ScholarPubMed
Kowlgi, GN, Giudicessi, JR, Brake, W, et al. Efficacy of intentional permanent atrial pacing in the long-term management of congenital long QT syndrome. J Cardiovasc Electrophysiol 2021; 32: 782789.CrossRefGoogle ScholarPubMed
Bellmann, B, Roser, M, Muntean, B, et al. Atrial standstill in sinus node disease due to extensive atrial fibrosis: impact on dual chamber pacemaker implantation. Europace 2016; 18: 238245.CrossRefGoogle ScholarPubMed
Ishikawa, T, Tsuji, Y, Makita, N. Inherited bradyarrhythmia: a diverse genetic background. J Arrhythm 2016; 32: 352358.CrossRefGoogle ScholarPubMed


McAlister, HF, Klementowicz, PT, Andrews, C, et al. Lyme carditis: an important cause of reversible heart block. Ann Intern Med 1989; 110: 339345.CrossRefGoogle Scholar
Forrester, JD, Mead, P. Third-degree heart block associated with lyme carditis: review of published cases. Clin Infect Dis 2014; 59: 9961000.CrossRefGoogle Scholar
Nunes, MCP, Beaton, A, Acquatella, H, et al. Chagas cardiomyopathy: an update of current clinical knowledge and management: a scientific statement from the American Heart Association. Circulation 2018; 138: 3341.CrossRefGoogle ScholarPubMed
Bocchi, EA, Bestetti, RB, Scanavacca, MI, et al. Chronic Chagas heart disease management: from etiology to cardiomyopathy treatment. J Am Coll Cardiol 2017; 70: 15101524.CrossRefGoogle ScholarPubMed
Dionne, A, Mah, D, Son, MF, et al. Atrio-ventricular block in children with multisystem inflammatory syndrome. Pediatrics 2020; 146: e2020009704.CrossRefGoogle Scholar
Batra, AS, Epstein, D, Silka, MJ. The clinical course of acquired complete heart block in children with acute myocarditis. Pediatr Cardiol 2003; 24: 495497.Google ScholarPubMed

Implantable cardioverter defibrillators: introduction

Cunningham, T, Roston, TM, Franciosi, S, et al. Initially unexplained cardiac arrest in children and adolescents: a national experience from the Canadian Pediatric Heart Rhythm Network. Heart Rhythm 2020; 17: 975981.CrossRefGoogle ScholarPubMed
Rucinski, C, Winbo, A, Marcondes, L, et al. A population-based registry of patients with inherited cardiac conditions and resuscitated cardiac arrest. J Am Coll Cardiol 2020; 75: 26982707.CrossRefGoogle ScholarPubMed
Silka, MJ, Kobayashi, RL, Hill, AC, et al. Pediatric survivors of out-of-hospital ventricular fibrillation: etiologies and outcomes. Heart Rhythm 2018; 15: 116121.CrossRefGoogle ScholarPubMed
van der Werf, C, Lieve, KV, Bos, JM, et al. Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest. Eur Heart J 2019; 40: 29532961.CrossRefGoogle ScholarPubMed
Cohen, MI, Etheridge, SP. Indications for implantable cardioverter defibrillator therapy, device and lead selection. In: Shah, M, Rhodes, L, Kaltman, J (eds). Cardiac Pacing and Defibrillation in Pediatric and Congenital Heart Disease. John Wiley and Sons Ltd, West Sussex, UK, 2017: 6290.CrossRefGoogle Scholar
Minier, M, Probst, V, Berthome, P, et al. Age at diagnosis of Brugada syndrome: influence on clinical characteristics and risk of arrhythmia. Heart Rhythm 2020; 17: 743749.CrossRefGoogle ScholarPubMed
Silka, MJ, Kron, J, Dunnigan, A, Dick, M. 2nd Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients. The Pediatric Electrophysiology Society. Circulation 1993; 87: 800807.CrossRefGoogle Scholar
Berul, CI, Van Hare, GF, Kertesz, NJ, et al. Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients. J Am Coll Cardiol 2008; 51: 16851691.CrossRefGoogle ScholarPubMed
Von Bergen, NH, Atkins, DL, Dick, M 2nd, et al. Multicenter study of the effectiveness of implantable cardioverter defibrillators in children and young adults with heart disease. Pediatr Cardiol 2011; 32: 399405.CrossRefGoogle Scholar
Baskar, S, Bao, H, Minges, KE, Spar, DS, et al. Characteristics and outcomes of pediatric patients who undergo placement of implantable cardioverter defibrillators: insights from the National Cardiovascular data registry. Circ Arrhythm Electrophysiol 2018; 11: e006542.CrossRefGoogle ScholarPubMed
Collins, KK, Schaffer, MS, Liberman, L, et al. Fascicular and nonfascicular left ventricular tachycardias in the young: an international multicenter study. J Cardiovasc Electrophysiol 2013; 24: 640648.CrossRefGoogle Scholar
Roggen, A, Pavlovic, M, Pfammatter, JP. Frequency of spontaneous ventricular tachycardia in a pediatric population. Am J Cardiol 2008; 101: 852854.CrossRefGoogle Scholar
Wu, J, Chen, Y, Ji, W, Gu, B, et al. Catheter ablation of ventricular tachycardia in the pediatric patients: a single-center experience. Pacing Clin Electrophysiol 2020; 43: 3746.CrossRefGoogle ScholarPubMed
Li, XM, Jiang, H, Li, YH, Zhang, Y, et al. Effectiveness of Radiofrequency Catheter ablation of outflow tract ventricular arrhythmias in children and adolescents. Pediatr Cardiol 2016; 37: 14751481.CrossRefGoogle ScholarPubMed
Sears, SF, Hazelton, AG, St Amant, J. Quality of life in pediatric patients with implantable cardioverter defibrillators. Am J Cardiol 2011; 107: 10231027.CrossRefGoogle ScholarPubMed
Kini, V, Soufi, MK, Deo, R, et al. Appropriateness of primary prevention implantable cardioverter-defibrillators at the time of generator replacement: are indications still met? J Am Coll Cardiol 2014; 63: 23882394.CrossRefGoogle Scholar
Schwartz, PJ, Spazzolini, C, Priori, SG, et al. Who are the long-QT syndrome patients who receive an implantable cardioverter-defibrillator and what happens to them?: data from the European Long-QT Syndrome Implantable Cardioverter-Defibrillator (LQTS ICD) Registry. Circulation 2010; 122: 12721282.CrossRefGoogle ScholarPubMed
Vincent, GM, Schwartz, PJ, Denjoy, I, et al. High efficacy of beta-blockers in long-QT syndrome type 1: contribution of noncompliance and QT-prolonging drugs to the occurrence of beta-blocker treatment “failures”. Circulation 2009; 119: 215221.CrossRefGoogle ScholarPubMed
Wedekind, H, Burde, D, Zumhagen, S, et al. QT interval prolongation and risk for cardiac events in genotyped LQTS-index children. Eur J Pediatr 2009; 168: 11071115.CrossRefGoogle ScholarPubMed
Schwartz, PJ, Priori, SG, Cerrone, M, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation 2004; 109: 18261833.CrossRefGoogle ScholarPubMed
Bos, JM, Bos, KM, Johnson, JN, et al. Left cardiac sympathetic denervation in long QT syndrome: analysis of therapeutic nonresponders. Circ Arrhythm Electrophysiol 2013; 6: 705711.CrossRefGoogle ScholarPubMed
Garson, Jr. , A, Dick, M 2nd, Fournier, A, et al. The long QT syndrome in children. An international study of 287 patients. Circulation 1993; 87: 18661872.CrossRefGoogle Scholar
Spazzolini, C, Mullally, J, Moss, AJ, et al. Clinical implications for patients with long QT syndrome who experience a cardiac event during infancy. J Am Coll Cardiol 2009; 54: 832837.CrossRefGoogle ScholarPubMed
Biton, Y, Rosero, S, Moss, AJ, et al. Primary prevention with the implantable cardioverter-defibrillator in high-risk long-QT syndrome patients. Europace 2019; 21: 339346.CrossRefGoogle ScholarPubMed
Moss, AJ, Zareba, W, Hall, WJ, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000; 101: 616623.CrossRefGoogle ScholarPubMed
Liu, JF, Jons, C, Moss, AJ, et al. Risk factors for recurrent syncope and subsequent fatal or near-fatal events in children and adolescents with long QT syndrome. J Am Coll Cardiol 2011; 57: 941950.CrossRefGoogle ScholarPubMed
Goldenberg, I, Moss, AJ, Peterson, DR, et al. Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 2008; 117: 21842191.CrossRefGoogle ScholarPubMed
Giudicessi, JR, Ackerman, MJ. Genotype- and phenotype-guided management of congenital long QT syndrome. Curr Probl Cardiol 2013; 38: 417455.CrossRefGoogle ScholarPubMed
Dufendach, KA, Timothy, K, Ackerman, MJ, et al. Clinical outcomes and modes of death in Timothy syndrome: a multicenter international study of a rare disorder. JACC Clin Electrophysiol 2018; 4: 459466.CrossRefGoogle Scholar
Crotti, L, Spazzolini, C, Tester, DJ, et al. Calmodulin mutations and life-threatening cardiac arrhythmias: insights from the International Calmodulinopathy registry. Eur Heart J 2019; 40: 29642975.CrossRefGoogle ScholarPubMed
Mazzanti, A, Maragna, R, Vacanti, , et al. Interplay between genetic substrate, QTc duration, and arrhythmia risk in patients with long QT syndrome. J Am Coll Cardiol 2018; 71: 16631671.CrossRefGoogle ScholarPubMed
Etheridge, SP, Sanatani, S, Cohen, MI, Albaro, CA, Saarel, EV, Bradley, DJ. Long QT syndrome in children in the era of implantable defibrillators. J Am Coll Cardiol 2007; 50: 13351340.CrossRefGoogle ScholarPubMed
Moore, JP, Gallotti, RG, Shannon, KM, et al. Genotype predicts outcomes in fetuses and neonates with severe congenital long QT syndrome. JACC Clin Electrophysiol 2020; 6: 15611570.CrossRefGoogle ScholarPubMed
Roston, TM, Jones, K, Hawkins, NM, et al. Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review. Heart Rhythm 2018; 15: 17911799.CrossRefGoogle ScholarPubMed
Miyake, CY, Webster, G, Czosek, RJ, et al. Efficacy of implantable cardioverter defibrillators in young patients with catecholaminergic polymorphic ventricular tachycardia: success depends on substrate. Circ Arrhythm Electrophysiol 2013; 6: 579587.CrossRefGoogle ScholarPubMed
Priori, S, Napolitano, C, Memmi, M, et al. Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation 2002; 106: 6974.CrossRefGoogle ScholarPubMed
Roston, TM, Haji-Ghassemi, O, LaPage, MJ, et al. Catecholaminergic polymorphic ventricular tachycardia patients with multiple genetic variants in the PACES CPVT registry. PLoS One 2018; 13: e0205925.CrossRefGoogle ScholarPubMed
Hayashi, M, Denjoy, I, Extramiana, F, et al. Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia. Circulation 2009; 119: 24262434.CrossRefGoogle ScholarPubMed
Kannankeril, PJ, Moore, JP, Cerrone, M, et al. Efficacy of Flecainide in the treatment of catecholaminergic polymorphic ventricular tachycardia: a randomized clinical trial. JAMA Cardiol 2017; 2: 759766.CrossRefGoogle ScholarPubMed
De Ferrari, GM, Dusi, V, Spazzolini, C, et al. Clinical management of catecholaminergic polymorphic ventricular tachycardia: the role of left cardiac sympathetic denervation. Circulation 2015; 131: 21852193.CrossRefGoogle ScholarPubMed
Olde Nordkamp, LR, Postema, PG, Knops, RE. Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: a systematic review and meta-analysis of inappropriate shocks and complications. Heart Rhythm 2016; 13: 443454.CrossRefGoogle ScholarPubMed
Roses-Noguer, F, Jarman, JW, Clague, JR, et al. Outcomes of defibrillator therapy in catecholaminergic polymorphic ventricular tachycardia. Heart Rhythm 2014; 11: 5866.CrossRefGoogle ScholarPubMed
Gonzalez Corcia, MC, de Asmundis, C, Chierchia, GB, et al. Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management. Cardiol Young 2016; 26: 10441055.CrossRefGoogle ScholarPubMed
Gonzalez Corcia, MC, Sieira, J, Sarkozy, A, et al. Brugada syndrome in the young: an assessment of risk factors predicting future events. Europace 2017; 19: 18641873.Google Scholar
Gonzalez Corcia, MC, Sieira, J, Pappaert, G, et al. A clinical score model to predict lethal events in young patients (≤19 years) with the Brugada syndrome. Am J Cardiol 2017; 120: 797802.CrossRefGoogle Scholar
Probst, V, Denjoy, I, Meregalli, PG, et al. Clinical aspects and prognosis of Brugada syndrome in children. Circulation 2007; 115: 20422048.CrossRefGoogle ScholarPubMed
Michowitz, Y, Milman, A, Andorin, A, et al. Characterization and management of arrhythmic events in young patients with Brugada syndrome. J Am Coll Cardiol 2019; 73: 17561765.CrossRefGoogle ScholarPubMed
Gonzalez Corcia, MC, Sieira, J, et al. Implantable cardioverter-defibrillators in children and adolescents with Brugada syndrome. J Am Coll Cardiol 2018; 71: 148157.CrossRefGoogle ScholarPubMed
Andorin, A, Behr, R, Denjoy, I, et al. Impact of clinical and genetic findings on the management of young patients with Brugada syndrome. Heart Rhythm 2016; 13: 12741282.CrossRefGoogle ScholarPubMed
Maron, BJ, Rowin, EJ, Casey, SA, et al. Hypertrophic cardiomyopathy in children, adolescents, and young adults associated with low cardiovascular mortality with contemporary management strategies. Circulation 2016; 133: 6273.CrossRefGoogle Scholar
Maron, BJ, Spirito, P, Ackerman, MJ, et al. Prevention of sudden cardiac death with implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol 2013; 61: 15271535.CrossRefGoogle ScholarPubMed
Miron, A, Lafreniere-Roula, M, Steve Fan, CP, et al. A validated model for sudden cardiac death risk prediction in pediatric hypertrophic cardiomyopathy. Circulation 2020; 142: 217229.CrossRefGoogle ScholarPubMed
Norrish, G, Cantarutti, N, Pissaridou, E, et al. Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: a systematic review and meta-analysis. Eur J Prev Cardiol 2017; 24: 12201230.CrossRefGoogle ScholarPubMed
Balaji, S, DiLorenzo, MP, Fish, FA, et al. Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: an international multicenter study. Heart Rhythm 2019; 16: 14621467.CrossRefGoogle Scholar
Norrish, G, Ding, T, Field, E, et al. Development of a novel risk prediction model for sudden cardiac death in childhood hypertrophic cardiomyopathy (HCM Risk-Kids). JAMA Cardiol 2019; 1: 918927.CrossRefGoogle Scholar
Kaski, J, Tomé Esteban, MT, Lowe, M. Outcomes after implantable cardioverter-defibrillator treatment in children with hypertrophic cardiomyopathy. Heart 2007; 93: 372374.CrossRefGoogle ScholarPubMed
Briasoulis, A, Mallikethi-Reddy, S, Palla, M, et al. Myocardial fibrosis on cardiac magnetic resonance and cardiac outcomes in hypertrophic cardiomyopathy: a meta-analysis. Heart 2015; 101: 14061411.CrossRefGoogle ScholarPubMed
Prinz, C, Schwarz, M, Ilic, I, et al. Myocardial fibrosis severity on cardiac magnetic resonance imaging predicts sustained arrhythmic events in hypertrophic cardiomyopathy. Can J Cardiol 2013; 29: 358363.CrossRefGoogle ScholarPubMed
Vermeer, AMC, Clur, SB, Blom, NA, et al. Penetrance of hypertrophic cardiomyopathy in children who are mutation positive. J Pediatr 2017; 188: 9195.CrossRefGoogle ScholarPubMed
Spinner, JA, Noel, CV, Denfield, SW, et al. Association of late gadolinium enhancement and degree of left ventricular hypertrophy assessed on cardiac magnetic resonance imaging with ventricular tachycardia in children with hypertrophic cardiomyopathy. Am J Cardiol 2016; 117: 13421348.CrossRefGoogle ScholarPubMed
Maron, BJ, Maron, MS, Semsarian, C. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol 2012; 60: 705715.CrossRefGoogle ScholarPubMed
Muchtar, E, Blauwet, LA, Gertz, MA. Restrictive cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, therapy. Circulation Research 2017; 121: 819837.CrossRefGoogle Scholar
Walsh, MA, Grenier, MA, Jeffries, LA, et al. Conduction abnormalities in pediatric patients with restrictive cardiomyopathies. Circ Heart Fail 2012; 5: 267273.CrossRefGoogle Scholar
Webber, SA, Lipshultz, SE, Sleeper, LA, et al. Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype: a report from the Pediatric Cardiomyopathy registry. Circulation 2012; 126: 12371244.CrossRefGoogle ScholarPubMed
Wittekind, SG, Ryan, TD, Gao, Z, et al. Contemporary outcomes of pediatric restrictive cardiomyopathy: a single-center experience. Pediatr Cardiol 2019; 40: 694704.CrossRefGoogle ScholarPubMed
Zangwill, SD, Naftel, D, L’Ecuyer, T, et al. Outcomes of children with restrictive cardiomyopathy listed for heart transplant: a multi-institutional study. J Heart Lung Transplant 2009; 28: 13351340.CrossRefGoogle ScholarPubMed
DeWitt, ES, Chandler, SF, Hylind, RJ, et al. Phenotypic manifestations of arrhythmogenic cardiomyopathy in children and adolescents. J Am Coll Cardiol 2019; 74: 346358.CrossRefGoogle ScholarPubMed
Mazzanti, A, Ng, K, Faragli, A, et al. Arrhythmogenic right ventricular cardiomyopathy: clinical course and predictors of arrhythmic risk. J Am Coll Cardiol 2016; 68: 25402550.CrossRefGoogle ScholarPubMed
Te Riele, A, James, CA, Sawant, AC, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy in the pediatric population: clinical characterization and comparison with adult-onset disease. JACC Clin Electrophysiol 2015; 1: 551560.CrossRefGoogle ScholarPubMed
Orgeron, GM, James, CA, Te Riele, A, et al. Implantable cardioverter-defibrillator therapy in arrhythmogenic right ventricular dysplasia/cardiomyopathy: predictors of appropriate therapy, outcomes, and complications. J Am Heart Assoc 2017; 6: e006242.CrossRefGoogle Scholar
Ortiz-Genga, MF, Cuenca, S, Dal Ferro, M, et al. Truncating FLNC mutations are associated with high-risk dilated and arrhythmogenic cardiomyopathies. J Am Coll Cardiol 2016; 68: 24402451.CrossRefGoogle ScholarPubMed
Dubin, AM, Berul, CI, Bevilacqua, LM, et al. The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation. J Card Fail 2003; 9: 375379.CrossRefGoogle ScholarPubMed
Bharucha, T, Lee, KJ, Daubeney, PE, et al. Sudden death in childhood cardiomyopathy: results from a long-term national population-based study. J Am Coll Cardiol 2015; 65: 23022310.CrossRefGoogle ScholarPubMed
Pahl, E, Sleeper, LA, Canter, CE, et al. PCMR (Pediatric Cardiomyopathy Registry) Investigators. Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy: a report from the Pediatric Cardiomyopathy registry. J Am Coll Cardiol 2012; 59: 607615.CrossRefGoogle ScholarPubMed
Middlekauff, HR, Stevenson, WG, Stevenson, LW, Saxon, LA. Syncope in advanced heart failure: high risk of sudden death regardless of origin of syncope. J Am Coll Cardiol 1993; 21: 110116.CrossRefGoogle ScholarPubMed
Bardy, GH, Lee, KL, Mark, DB, et al. Amiodarone or an implantable cardioverter-defibrillator for congestive heart failure. N Engl J Med 2005; 352: 225237.CrossRefGoogle ScholarPubMed
El-Assaad, I, Al-Kindi, SG, Oliveira, G, et al. Implantable cardioverter-defibrillator and wait-list outcomes in pediatric patients awaiting heart transplantation. Heart Rhythm 2015; 12: 24432448.CrossRefGoogle ScholarPubMed
Rhee, EK, Canter, CE, Basile, S, et al. Sudden death prior to pediatric heart transplantation: would implantable defibrillators improve outcome? J Heart Lung Transplant 2007; 26: 447452.CrossRefGoogle ScholarPubMed
Jefferies, JL, Wilkinson, JD, Sleeper, LA, et al. Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: results from the Pediatric Cardiomyopathy registry. J Card Fail 2015; 21: 877884.CrossRefGoogle ScholarPubMed
Brescia, ST, et al. Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center. Circulation 2013; 127: 22022208.CrossRefGoogle ScholarPubMed
van Waning, JI, Caliskan, K, Hoedemaekers, YM, et al. Genetics, clinical features, and long-term outcome of noncompaction cardiomyopathy. J Am Coll Cardiol 2018; 71: 711722.CrossRefGoogle ScholarPubMed
Khairy, P, Harris, L, Landzberg, MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation 2008; 117: 363370.CrossRefGoogle ScholarPubMed
Silka, MJ, Hardy, BG, Menashe, VD, et al. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects. J Am Coll Cardiol 1998; 32: 245251.CrossRefGoogle ScholarPubMed
Nieminen, HP, Jokinen, EV, Sairanen, HI. Causes of late deaths after pediatric cardiac surgery: a population-based study. J Am Coll Cardiol 2007; 50: 12631271.CrossRefGoogle ScholarPubMed
Miyazaki, A, Sakaguchi, H, Ohuchi, H, et al. Efficacy of hemodynamic-based management of tachyarrhythmia after repair of tetralogy of Fallot. Circ J 2012; 76: 28552862.CrossRefGoogle ScholarPubMed
Zeppenfeld, K, Schalij, MJ, Bartelings, MM, et al. Catheter ablation of ventricular tachycardia after repair of congenital heart disease: electroanatomic identification of the critical right ventricular isthmus. Circulation 2007; 116: 22412252.CrossRefGoogle ScholarPubMed
Triedman, JK. Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD? Implantable cardioverter defibrillator implantation guidelines based solely on left ventricular ejection fraction do not apply to adults with congenital heart disease. Circ Arrhythm Electrophysiol 2008; 1: 307316.CrossRefGoogle Scholar
Silka, MJ, Bar-Cohen, Y. Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD? Patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% should undergo prophylactic implantation of an implantable cardioverter defibrillator. Circ Arrhythm Electrophysiol 2008; 1: 298306.CrossRefGoogle Scholar
Jordan, CP, Freedenberg, V, Wang, Y, et al. Implant and clinical characteristics for pediatric and congenital heart patients in the national cardiovascular data registry implantable cardioverter defibrillator registry. Circ Arrhythm Electrophysiol 2014; 7: 10921100.CrossRefGoogle ScholarPubMed
Dechert, BE, Bradley, DJ, Serwer, GA, et al. Implantable cardioverter defibrillator outcomes in pediatric and congenital heart disease: time to system revision. Pacing Clin Electrophysiol 2016; 39: 703708.CrossRefGoogle ScholarPubMed
Krause, U, Müller, MJ, Wilberg, Y, et al. Transvenous and non-transvenous implantable cardioverter-defibrillators in children, adolescents, and adults with congenital heart disease: who is at risk for appropriate and inappropriate shocks? Europace 2019; 21: 106113.CrossRefGoogle ScholarPubMed
Kalra, Y, Radbill, A, Johns, JA, et al. Antitachycardia pacing reduces appropriate and inappropriate shocks in children and congenital heart disease patients. Heart Rhythm 2012; 9: 18291834.CrossRefGoogle ScholarPubMed
Sandhu, A, Ruckdeschel, E, Sauer, WH, et al. Perioperative electrophysiology study in patients with tetralogy of Fallot undergoing pulmonary valve replacement will identify those at high risk of subsequent ventricular tachycardia. Heart Rhythm 2018; 15: 679685.CrossRefGoogle ScholarPubMed
Alexander, ME, Walsh, EP, Saul, JP, Epstein, MR, Triedman, JK. Value of programmed ventricular stimulation in patients with congenital heart disease. J Cardiovasc Electrophysiol 1999; 10: 10331044.CrossRefGoogle ScholarPubMed
Khairy, P, Landzberg, MJ, Gatzoulis, MA, et al. Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study. Circulation 2004; 109: 19942000.CrossRefGoogle ScholarPubMed
Radbill, AE, Triedman, JK, Berul, CI, et al. System survival of nontransvenous implantable cardioverter-defibrillators compared to transvenous implantable cardioverter-defibrillators in pediatric and congenital heart disease patients. Heart Rhythm 2010; 7: 193198.CrossRefGoogle ScholarPubMed
von Alvensleben Johannes, C, Dechert, Brynn, Bradley David, J, et al. Subcutaneous implantable cardioverter-defibrillators in pediatrics and congenital heart disease: a pediatric and congenital electrophysiology society multicenter review. JACC Clin Electrophysiol 2020; 6: 17521761.CrossRefGoogle Scholar
Steinberg, JS, Varma, N, Cygankiewicz, I, et al. 2017 ISHNE-HRS expert consensus statement on ambulatory ECG and external cardiac monitoring/telemetry. Heart Rhythm 2017; 14: e55e96.CrossRefGoogle ScholarPubMed
Moya, A, Sutton, R, Ammirati, F, et al. Guidelines for the diagnosis and management of syncope. Eur Heart J 2009; 30: 26312671.Google ScholarPubMed
Macinnes, M, Martin, N, Fulton, H, McLeod, KA. Comparison of a smartphone-based ECG recording system with a standard cardiac event monitor in the investigation of palpitations in children. Arch Dis Child 2019; 104: 4347.CrossRefGoogle ScholarPubMed
Pradhan, S, Robinson, JA, Shivapour, J, et al. Ambulatory arrhythmia detection with ZIO® XT patch in pediatric patients: a comparison of devices. Pediatr Cardiol 2019; 40: 921924.CrossRefGoogle ScholarPubMed
Babikar, A, Hynes, B, Ward, N, et al. A retrospective study of the clinical experience of the implantable loop recorder in a paediatric setting. Int J Clin Pract 2008; 62: 15201525.CrossRefGoogle Scholar
Bezzerides, VJ, Walsh, A, Martuscello, M, et al. The real-world utility of the LINQ implantable loop recorder in pediatric and adult congenital heart patients. JACC Clin Electrophysiol 2019; 5: 245251.CrossRefGoogle Scholar
Placidi, S, Drago, F, Milioni, M, et al. Miniaturized implantable loop recorder in small patients: an effective approach to the evaluation of subjects at risk of sudden death. Pacing Clin Electrophysiol 2016; 39: 669674.CrossRefGoogle ScholarPubMed
Avari Silva, JN, Bromberg, BI, Emge, FK, et al. Implantable loop recorder monitoring for refining management of children with inherited arrhythmia syndromes. J Am Heart Assoc 2016; 5: e003632.CrossRefGoogle ScholarPubMed
Brignole, M, Moya, A, de Lange, FJ, et al. 2018 ESC Guidelines for the diagnosis and management of syncope. Eur Heart J 2018; 39: 18831948.CrossRefGoogle ScholarPubMed
Brignole, M, Vardas, P, Hoffman, E, et al. Indications for the use of diagnostic implantable and external ECG loop recorders. Europace 2009; 11: 671687.Google ScholarPubMed
Rossano, J, Bloemers, B, Sreeram, N, et al. Efficacy of implantable loop recorders in establishing symptom-rhythm correlation in young patients with syncope and palpitations. Pediatrics 2003; 112: e228e233.CrossRefGoogle ScholarPubMed
Edvardsson, N, Garutti, C, Rieger, G, et al. Unexplained syncope: implications of age and gender on patient characteristics and evaluation, the diagnostic yield of an implantable loop recorder, and the subsequent treatment. Clin Cardiol 2014; 37: 618625.CrossRefGoogle ScholarPubMed
Al Dhahri, KN, Potts, JE, Chiu, CC, et al. Are implantable loop recorders useful in detecting arrhythmias in children with unexplained syncope? PacingClin Electrophysiol 2009; 32: 14221427.Google Scholar
Frangini, PA, Cecchin, F, Jordao, L, et al. How revealing are insertable loop recorders in pediatrics? Pacing Clin Electrophysiol 2008; 31: 338343.CrossRefGoogle Scholar
Kenny, D, Chakrabarti, S, Ranasinghe, A, et al. Single-centre use of implantable loop recorders in patients with congenital heart disease. Europace 2009; 11: 303307.CrossRefGoogle Scholar
Serdyuk, S, Davtyan, K, Burd, S, et al. Cardiac arrhythmias and sudden unexpected death in epilepsy: results of long-term monitoring. Heart Rhythm 2021; 18: 221228.CrossRefGoogle ScholarPubMed
Bongiorni, MG, Burri, H, Deharo, JC, et al. 2018 EHRA expert consensus statement on lead extraction: recommendations on definitions, endpoints, research trial design, and data collection requirements for clinical scientific studies and registries: endorsed by APHRS/HRS/LAHRS. Europace 2018; 20: 1217.CrossRefGoogle ScholarPubMed
Fu, HX, Huang, XM, Zhong, L, et al. Outcome and management of pacemaker-induced superior vena cava syndrome. Pacing Clin Electrophysiol 2014; 37: 14701476.CrossRefGoogle ScholarPubMed
Riley, RF, Petersen, SE, Ferguson, JD, Bashir, Y. Managing superior vena cava syndrome as a complication of pacemaker implantation: a pooled analysis of clinical practice. Pacing Clin Electrophysiol 2010; 33: 420425.CrossRefGoogle ScholarPubMed
Atallah, J, Erickson, CC, Cecchin, F, et al. Multi-institutional study of implantable defibrillator lead performance in children and young adults: results of the Pediatric Lead Extractability and Survival Evaluation (PLEASE) study. Circulation 2013; 127: 23932402.CrossRefGoogle Scholar
Cecchin, F, Atallah, J, Walsh, EP, et al. Lead extraction in pediatric and congenital heart disease patients. Circ Arrhythmia Electrophysiol 2010; 3: 437444.CrossRefGoogle ScholarPubMed
Mah, DY, Prakash, A, Porras, D, et al. Coronary artery compression from epicardial leads: more common than we think. Heart Rhythm 2018; 15: 14391447.CrossRefGoogle ScholarPubMed
Fender, EA, Killu, AM, Cannon, BC, et al. Lead extraction outcomes in patients with congenital heart disease. Europace 2017; 19: 441446.Google ScholarPubMed
McCanta, AC, Kong, MH, Carboni, MP, et al. Laser lead extraction in congenital heart disease: a case-controlled study. Pacing Clin Electrophysiol 2013; 36: 372380.CrossRefGoogle ScholarPubMed
Moak, JP, Freedenberg, V, Ramwell, C, et al. Effectiveness of excimer laser-assisted pacing and ICD lead extraction in children and young adults. Pacing Clin Electrophysiol 2006; 29: 461466.CrossRefGoogle ScholarPubMed
El-Chami, MF, Sayegh, MN, Patel, A, et al. Outcomes of lead extracton in young adults. Heart Rhythm 2017; 14: 537540.CrossRefGoogle Scholar
Gourraud, JB, Chaix, MA, Shohoudie, A, et al. Transvenous lead extraction in adults with congenital heart disease: insights from a 20-year single-center experience. Circ Arrhythm Electrophysiol 2018; 11: e005409.CrossRefGoogle ScholarPubMed
Berul, CI, Villafane, J, Atkins, DL, et al. Pacemaker lead prolapse through the pulmonary valve in children. Pacing Clin Electrohysiol 2007; 30: 11831189.CrossRefGoogle ScholarPubMed
Webster, RG, Margossian, R, Alexander, ME, Cecchin, F, Triedman, JK, Walsh, EP, Berul, CI. Impact of transvenous ventricular pacing leads on tricuspid regurgitation in children and congenital heart disease patients. J Interv Card Electrophysiol 2008; 21: 6568.CrossRefGoogle ScholarPubMed
Bar-Cohen, Y, Berul, CI, Alexander, ME, et al. Age, size, and lead factors alone do not predict venous obstruction in children and young adults with transvenous lead systems. J Cardiovasc Electrophysiol 2006; 17: 754759.CrossRefGoogle ScholarPubMed
Joy, PS, Kumar, G, Poole, JE, et al. Cardiac implantable electronic device infections: who is at greatest risk? Heart Rhythm 2017; 14: 839.CrossRefGoogle ScholarPubMed
Sohal, M, Williams, S, Akhtar, M, et al. Laser lead extraction to facilitate cardiac implantable electronic device upgrade and revision in the presence of central venous obstruction. Europace 2014; 16: 8187.CrossRefGoogle ScholarPubMed
Gula, LJ, Ames, A, Woodburn, A, et al. Central venous occlusion is not an obstacle to device upgrade with the assistance of laser extraction. Pacing Clin Electrophysiol 2005; 28: 661666.CrossRefGoogle Scholar
Viganego, F, O’Donoghue, S, Eldadah, Z, et al. Effect of early diagnosis and treatment with percutaneous lead extraction on survival in patients with cardiac device infections. Am J Cardiol 2012; 109: 14661471.CrossRefGoogle ScholarPubMed
Baddour, LM, Epstein, AE, Erickson, CC, et al. Update on cardiovascular implantable electronic device infections and their management: a scientific statement from the American Heart Association. Circulation 2010; 121: 458477.CrossRefGoogle ScholarPubMed
Janson, CM, Patel, AR, Bonney, WJ, et al. Implantable cardioverter-defibrillator lead failure in children and young adults, a matter of lead diameter of lead design? J Am Coll Cardiol 2014; 63: 133140.CrossRefGoogle Scholar
Escudero, CA, Mah, DY, Miyake, CY, et al. Riata lead failure in pediatric and congenital heart disease patients. J Cardiovasc Electrophysiol 2019; 30: 320325.CrossRefGoogle ScholarPubMed
Slotwiner, D, Varma, N, Akar, J, et al. HRS Expert Consensus Statement on remote interrogation and monitoring for cardiovascular implantable electronic devices. Heart Rhythm 2015; 12: E69E100.CrossRefGoogle ScholarPubMed
Wilkoff, BL, Auricchio, A, Brugada, J, et al. HRS/EHRA expert consensus on the monitoring of cardiovascular implantable electronic devices (CIEDs): description of techniques, indications, personnel, frequency and ethical considerations. Heart Rhythm 2008; 5: 907925.CrossRefGoogle ScholarPubMed
Dasgupta, S, Madani, S, Figueroa, R, et al. Myocardial deformation as a predictor of right ventricular pacing -induced cardiomyopathy in the pediatric population. J Cardiovasc Electrophysiol 2020; 31: 337344.CrossRefGoogle ScholarPubMed
Song, MK, Kim, NY, Bae, EJ, et al. Long term follow up of epicardial pacing and left ventricular dysfunction in children with congenital heart block. Ann Thorac Surg 2020; 109: 19131920.CrossRefGoogle ScholarPubMed
Gebauer, RA, Tomek, V, Salameh, A. Predictors of left ventricular remodelling and failure in right ventricular pacing in the young. Eur Heart J 2009; 30: 10971104.CrossRefGoogle Scholar
Thambo, JP, Bordachar, P, Garrigue, S, et al. Detrimental ventricular remodelling in patients with congenital complete heart block and chronic right ventricular apical pacing. Circulation 2004; 110: 37663772.CrossRefGoogle ScholarPubMed
Tantengco, MV, Thomas, RL, Karpawich, PP. Left ventricular dysfunction after long-term right ventricular apical pacing in the young. J Am Coll Cardiol 2001; 37: 20932100.CrossRefGoogle Scholar
Gonzalez Corcia, MC, Remy, LS, Marchandise, S, et al. Exercise performance in young patients with complete atrioventricular block: the relevance of synchronous atrioventricular pacing. Cardiol Young 2016: 261066261071.Google ScholarPubMed
Ross, B, Zeigler, V, Zinner, A, et al. The effect of exercise on the atrial electrogram voltage in young patients. Pacing Clin Electrophysiol 1991; 4: 20922097.CrossRefGoogle Scholar
Chudzik, M, Klimczak, A, Wranicz, JK. Ambulatory Holter monitoring in asymptomatic patients with DDD pacemakers—do we need ACC/AHA guideline revision? Arch Med Sci 2013; 9: 815820.CrossRefGoogle Scholar
Diemberger, I, Gardini, B, Martignani, C, et al. Holter ECG for pacemaker/defibrillator carriers: what is its role in the era of remote monitoring? Heart 2015; 101: 12721278.CrossRefGoogle ScholarPubMed
Dechert, BE, Sewer, GA, Bradley, DJ, et al. Cardiac implantable electronic device remote monitoring surveillance in pediatric and congenital heart disease: utility relative to frequency. Heart Rhythm 2015; 12: 117122.CrossRefGoogle ScholarPubMed
Malloy, LE, Gingerich, J, Olson, MD, et al. Remote monitoring of cardiovascular implantable devices in the pediatric population improves detection of adverse events. Pediatr Cardiol 2014; 35: 301306.CrossRefGoogle ScholarPubMed
Nishii, N, Miyoshi, A, Kubo, M, et al. Analysis of arrhythmic events is useful to detect lead failure earlier in patients followed by remote monitoring. J Cardiovasc Electrophysiol 2018; 29: 463470.CrossRefGoogle ScholarPubMed
Piccini, JP, Snell, J, Prillinger, JB, et al. Impact of remote monitoring on clinical events and associated health care utilization: a nationwide assessment. Heart Rhythm 2016; 13: 22792286.CrossRefGoogle ScholarPubMed
Hummel, JP, Leipold, RJ, Amorosi, SL, et al. Outcomes and costs of remote monitoring among patients with implanted cardiac defibrillators: an economic model based on the PREDICT RM database. J Cardiovasc Electrophysiol 2019; 30: 10661077.CrossRefGoogle ScholarPubMed
Dechert, BE, Sewer, GA, Bradley, DJ, et al. Frequency of CIED remote monitoring: a quality improvement follow-up study. Pacing Clin Electrophysiol 2019; 42: 959962.CrossRefGoogle ScholarPubMed
Boyer, SL, Silka, MJ, Bar-Cohen, Y. Current practices in the monitoring cardiac rhythm devices in pediatrics and congenital heart disease. Pediatr Cardiol 2015; 36: 821826.CrossRefGoogle Scholar
Kadish, AH, Buxton, AE, Kennedy, HL, et al. ACC/AHA clinical competence statement on electrocardiography and ambulatory electrocardiography: a report of the ACC/AHA/ACP-ASIM task force on clinical competence (ACC/AHA Committee to develop a clinical competence statement on electrocardiography and ambulatory electrocardiography) endorsed by the International Society for Holter and noninvasive electrocardiology. Circulation 2001; 104: 31693178.Google ScholarPubMed
Bricker, JT, Garson, A, Traweek, M, et al. The use of exercise testing in children to evaluate abnormalities of pacemaker function not apparent at rest. Pacing Clin Electrophysiol 1985; 8: 656660.CrossRefGoogle Scholar
Sampio, SMV, Craveiro, NM, Darrieux, F, et al. Accuracy of the pacemaker event recorder versus Holter-ECG to detect both symptomatic and asymptomatic ventricular arrhythmias. J Cardiovasc Electrophysiol 2018; 29: 154159.CrossRefGoogle Scholar
Munawar, DA, Chan, JEZ, Emami, M, et al. Magnetic resonance imaging in non-conditional pacemakers and implantable cardioverter-defibrillators: a systematic review and meta-analysis. Europace 2020; 22: 288298.CrossRefGoogle ScholarPubMed
Bireley, M, Kovach, JR, Morton, C, et al. Cardiac magnetic resonance imaging (MRI) in children is safe with most pacemaker systems, including those with epicardial leads. Pediatr Cardiol 2020; 41: 801808.CrossRefGoogle ScholarPubMed
Shah, AD, Morris, MA, Hirsh, DS, et al. Magnetic resonance imaging safety in nonconditional pacemaker and defibrillator recipients: a meta-analysis and systematic review. Heart Rhythm 2018; 15: 10011008.CrossRefGoogle ScholarPubMed
Gakenheimer-Smith, L, Etheridge, SP, Niu, MC, et al. MRI in pediatric and congenital heart disease patients with CIEDs and epicardial or abandoned leads. Pacing Clin Electrophysiol 2020; 43: 797804.CrossRefGoogle ScholarPubMed
Schaller, R, Brunker, T, Riley, MP, et al. Magnetic resonance imaging in patients with cardiac implantable electronic devices with abandoned leads. JAMA Cardiol 2021; 6: 549556.CrossRefGoogle ScholarPubMed
Nazarian, S, Hansford, R, Rahsepar, AA, et al. Safety of magnetic resonance imaging in patients with cardiac devices. N Engl J Med 2017; 377: 25552564.CrossRefGoogle ScholarPubMed
Padmanabhan, D, Kella, DK, Mehta, R, et al. Safety of magnetic resonance imaging in patients with legacy pacemakers and defibrillators and abandoned leads. Heart Rhythm 2018; 15: 228233.CrossRefGoogle ScholarPubMed
Rahsepar, AA, Zimmerman, SL, Hansford, R, et al. The relationship between MRI radiofrequency energy and function of nonconditional implanted cardiac devices: a prospective evaluation. Radiology 2020; 295: 307313.CrossRefGoogle ScholarPubMed
Balmer, C, Gass, M, Dave, H, et al. Magnetic resonance imaging of patients with epicardial leads: in vitro evaluation of temperature changes at the lead tip. J Interv Card Electrophysiol 2019; 56: 321326.CrossRefGoogle Scholar
Langman, DA, Goldberg, IB, Finn, JP, et al. Pacemaker lead tip heating in abandoned and pacemaker-attached leads at 1.5 Tesla MRI. J Magn Reson Imaging 2011; 33: 426431.CrossRefGoogle ScholarPubMed
Mattei, E, Calcagnini, G, Censi, F, et al. Role of the lead structure in MRI-induced heating: in vitro measurements on 30 commercial pacemaker/defibrillator leads. Magn Reson Med 2012; 67: 925935.CrossRefGoogle ScholarPubMed
Higgins, JV, Gard, JJ, Sheldon, SH, et al. Safety and outcomes of magnetic resonance imaging in patients with abandoned pacemaker and defibrillator leads. Pacing Clin Electrophysiol 2014; 37: 12841290.CrossRefGoogle ScholarPubMed
Saarel, EV, Pilcher, TA, Etheridge, SP. Safety of sports for pediatric and congenital ICD and pacemaker patients. Heart Rhythm 2013; 10 (5S): 211.Google Scholar
Lampert, R, Olshansky, B, Heidbuchel, H, et al. Safety of sports for athletes with implantable cardioverter-defibrillators: results of a prospective, multinational registry. Circulation 2013; 127: 20212030.CrossRefGoogle ScholarPubMed
Lampert, R, Olshansky, B, Heidbuchel, H, et al. Safety of sports for athletes with implantable cardioverter defibrillators: long-term results of a prospective multinational registry. Circulation 2017; 135: 23102312.CrossRefGoogle ScholarPubMed
Saarel, EV, Law, I, Berul, CI, et al. Safety of sports for young patients with implantable cardioverter-defibrillators: long-term results of a prospective multinational registry. Circ Arrhythm Electrophysiol 2018; 11: e006305.CrossRefGoogle Scholar
Maron, BJ, Udelson, JE, Bonow, RO, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis. Circulation 2015; 132: e273e280.CrossRefGoogle ScholarPubMed
Zipes, DP, Link, MS, Ackerman, MJ, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 9: arrhythmias and conduction defects. Circulation 2015; 132: e315e325.CrossRefGoogle ScholarPubMed
Maron, BJ, Zipes, DP. Introduction: eligibility recommendations for competitive athletes with cardiovascular abnormalities-general considerations. J Am Coll Cardiol 2005; 45: 13181321.CrossRefGoogle ScholarPubMed
Pelliccia, A, Fagard, R, Bjørnstad, HH, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2005; 26: 14221445.CrossRefGoogle Scholar
Mitchell, JH, Haskell, W, Snell, P, et al. Task Force 8: classification of sports. J Am Coll Cardiol 2005; 45: 13641367.CrossRefGoogle ScholarPubMed
Lampert, R, Cannom, D, Olshansky, B. Safety of sports participation in patients with implantable cardioverter defibrillators: a survey of heart rhythm society members. J Cardiovasc Electrophysiol 2006; 17: 1115.CrossRefGoogle ScholarPubMed
The World Bank. Country and Lending Groups, 2021. Retrieved January 2021, Scholar
Bonny, A, Mgantcha, M, Jeilan, M, et al. Statistics on the use of cardiac electronic devices and interventional electrophysiological procedures in Africa from 2011 to 2016: report of the Pan African Society of Cardiology (PASCAR) Cardiac Arrhythmias and Pacing Task Forces. Europace 2018; 1: 15131526.CrossRefGoogle Scholar
Murray, LE, Smith, AH, Flack, EC, et al. Genotypic and phenotypic predictors of complete heart block and recovery of conduction after surgical repair of congenital heart disease. Heart Rhythm 2017; 14: 402409.CrossRefGoogle ScholarPubMed
Paech, C, Dahnert, I, Kostelka, M, et al. Association of temporary complete AV block and junctional ectopic tachycardia after surgery for congenital heart disease. Ann Pediatr Cardiol 2015; 8: 1419.CrossRefGoogle ScholarPubMed
Batra, AS, Wells, WJ, Hinoki, KW, et al. Late recovery of atrioventricular conduction after pacemaker implantation for complete heart block associated with surgery for congenital heart disease. J Thorac Cardiovasc Surg 2003; 125: 12911293.CrossRefGoogle ScholarPubMed
Bruckheimer, E, Berul, CI, Kopf, GS, et al. Late recovery of surgically-induced atrioventricular block in patients with congenital heart disease. J Interv Card Electrophysiol 2002; 6: 191195.CrossRefGoogle ScholarPubMed
Khairy, T, Lupien, MA, Nava, S, et al. Infections associated with resterilized pacemakers and defibrillators. N Engl J Med 2020; 382: 18231831.CrossRefGoogle ScholarPubMed
Baman, TS, Meier, P, Romero, J, et al. Safety of pacemaker reuse: a meta-analysis with implications for underserved nations. Circ Arrhythm Electrophysiol 2011; 4: 318323.CrossRefGoogle ScholarPubMed
Kapoor, A, Vora, A, Nataraj, G, Mishra, S, et al. Guidance on reuse of cardio-vascular catheters and devices in India: a consensus document. Indian Heart J 2017; 69: 357363.CrossRefGoogle ScholarPubMed
Roberts, PR, El Refai, MH. The use of App-based follow-up of cardiac implantable electronic devices. Card Fail Rev 2020; 6: e03.CrossRefGoogle ScholarPubMed
Elwyn, G, Frosch, D, Thomson, R, et al. Shared decision making: a model for clinical practice. J Gen Intern Med 2012; 27: 13611367.CrossRefGoogle Scholar
Greenfield, S, Kaplan, S, Ware Jr. JE. Expanding patient involvement in care. Effects on patient outcomes. Ann Intern Med 1985; 102: 520528.CrossRefGoogle Scholar
Legare, F, Adekpedjou, R, Stacey, D, et al. Interventions for increasing the use of shared decision making by healthcare professionals. Cochrane Database Syst Rev 2018; 7: CD006732.CrossRefGoogle Scholar
Khairy, P, Dore, A, Poirier, N, et al. Risk stratification in surgically repaired tetralogy of Fallot. Expert Rev Cardiovasc Ther 2009; 7: 755762.CrossRefGoogle ScholarPubMed
Goette, A, Auricchio, A, Boriani, G, et al. EHRA White paper: knowledge gaps in arrhythmia management—status 2019. Europace 2019; 21: 993994.CrossRefGoogle Scholar
Burns, KM, Evans, F, Kaltman, JR. Pediatric ICD utilization in the United States from 1997–2006. Heart Rhythm 2011; 8: 2328.CrossRefGoogle Scholar
Breatnach, CR, Dunne, L, Al-Alawi, K, et al. Leadless Micra pacemaker use in the pediatric population: device implantation and short-term outcomes. Pediatr Cardiol 2020; 41: 683686.CrossRefGoogle ScholarPubMed
Lyon, S, Dandamudi, G, Kean, AC. Permanent His-bundle pacing in pediatrics and congenital heart disease. J Innov Card Rhythm Manag 2020; 11: 40054012.CrossRefGoogle Scholar
Figure 0

Table 1. Guidelines, expert consensus statements, and reports Cited

Figure 1

Table 2. Class of Recommendation and Level of Evidence Categories*

Supplementary material: File

Shah et al. supplementary material

Shah et al. supplementary material 1