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2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients

Open AccessPublished:July 29, 2021DOI:https://doi.org/10.1016/j.hrthm.2021.07.038

      Abstract:

      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.

      Keywords

      Abbreviations:

      ACM (arrhythmogenic cardiomyopathy), ARVC (arrhythmogenic right ventricular cardiomyopathy), AV (atrioventricular), BrS (Brugada syndrome), CCAVB (congenital complete atrioventricular block), CHD (congenital heart disease), CIED (cardiovascular implantable electronic device), COR (class of recommendation), CPVT (catecholaminergic polymorphic ventricular tachycardia), ECG (electrocardiogram), HCM (hypertrophic cardiomyopathy), ICD (implantable cardioverter defibrillator), ICM (insertable cardiac monitor), IPE (in-person evaluation), LGE (late gadolinium enhancement), LVEF (left ventricular ejection fraction), LMIC (low- and middle-income countries), LOE (level of evidence), LQTS (long QT syndrome), MRI (magnetic resonance imaging), NIDCM (nonischemic dilated cardiomyopathy), RCT (randomized clinical trial), RIM (remote interrogation and monitoring), SCA (sudden cardiac arrest), SCD (sudden cardiac death), SND (sinus node dysfunction), TTM (transtelephonic monitoring), VF (ventricular fibrillation), VT (ventricular tachycardia)
      Document Reviewers: Philip M. Chang, MD, FHRS, FACC; Fabrizio Drago, MD, FAIAC; Anne M. Dubin, MD, FHRS; Susan P. Etheridge, MD, FHRS; Apichai Kongpatanayothin, MD, FAsCC; Jose Manuel Moltedo, MD, FACC; Ashish A. Nabar, MD, DNB, PhD, FISE; George F. Van Hare, MD, FHRS, FACC, FAAP

      Preamble

      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.
      • Frye R.L.
      • Collins J.J.
      • DeSanctis R.W.
      • 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).
      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,”
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      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.
      • Levine G.N.
      • O’Gara P.T.
      • Beckman J.A.
      • 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.
      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).

      1. Introduction

      1.1 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;
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      • Levine G.N.
      • O’Gara P.T.
      • Beckman J.A.
      • 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.
      • Halperin J.L.
      • Levine G.N.
      • Al-Khatib S.M.
      • 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.
      • Kusumoto F.M.
      • 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.
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      • 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.
      • Khairy P.
      • Van Hare G.F.
      • Balaji S.
      • et al.
      2014 PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease.
      • 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).
      • Shen W.K.
      • Sheldon R.S.
      • Benditt D.G.
      • et al.
      2017 ACC/AHA/HRS guidelines for the evaluation and management of patients with syncope.
      • Al-Khatib S.M.
      • Stevenson W.G.
      • Ackerman M.J.
      • et al.
      2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death.
      • Priori S.G.
      • 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.
      • Priori S.G.
      • Wilde A.A.
      • Horie M.
      • et al.
      2013 HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes.
      • Ommen S.R.
      • Mital S.
      • Burke M.A.
      • 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.
      • Towbin J.A.
      • McKenna W.J.
      • Abrams D.J.
      • et al.
      2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.
      • Kirk R.
      • Dipchand A.I.
      • Rosenthal D.N.
      • et al.
      2014 The International Society for Heart and Lung Transplantation guidelines for the management for pediatric heart failure.
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • Wilkoff B.L.
      • et al.
      2017 HRS expert consensus statement on cardiovascular implantable electronic device lead management and extraction.
      • Indik J.H.
      • Gimbel J.R.
      • Abe H.
      • et al.
      2017 HRS expert consensus statement on magnetic resonance imaging and radiation exposure in patients with cardiovascular implantable electronic devices.
      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 1Guidelines, Expert Consensus Statements, and Reports Cited
      TitleOrganizationYear (reference)
      Guidelines for permanent pacemaker implantationACC/AHA1984 (
      • Frye R.L.
      • Collins J.J.
      • DeSanctis R.W.
      • 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).
      )
      Guidelines for the management of patients with bradycardia and cardiac conduction delayACC/AHA/HRS2019 (
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      )
      Report: Innovations, modifications, and evolution of clinical practice guidelinesACC/AHA2019 (
      • Levine G.N.
      • O’Gara P.T.
      • Beckman J.A.
      • 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.
      )
      Report: Evolution of the clinical practice guideline recommendation classification systemACC/AHA2016 (
      • Halperin J.L.
      • Levine G.N.
      • Al-Khatib S.M.
      • 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.
      )
      ECS: ICD therapy in patients who are not included or not well represented in clinical trialsHRS/ACC/AHA2014 (
      • Kusumoto F.M.
      • 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.
      )
      Guidelines for device-based therapyACC/AHA/HRS2008 (
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      )
      Update of the 2008 device-based therapy guidelinesACC/AHA/HRS2012 (
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      )
      ECS: Arrhythmias in congenital heart diseaseEHRA/AEPC/ESC2018 (
      • 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.
      )
      ECS: Recognition and management of arrhythmias in adult congenital heart diseasePACES/HRS2014 (
      • Khairy P.
      • Van Hare G.F.
      • Balaji S.
      • et al.
      2014 PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease.
      )
      Guidelines on cardiac pacing and resynchronizationESC2013 (
      • 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).
      )
      Guidelines for the evaluation and management of patients with syncopeACC/AHA/HRS2017 (
      • Shen W.K.
      • Sheldon R.S.
      • Benditt D.G.
      • et al.
      2017 ACC/AHA/HRS guidelines for the evaluation and management of patients with syncope.
      )
      Guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac deathAHA/ACC/HRS2018 (
      • Al-Khatib S.M.
      • Stevenson W.G.
      • Ackerman M.J.
      • et al.
      2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death.
      )
      Guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac deathESC2015 (
      • Priori S.G.
      • 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.
      )
      ECS: The diagnosis and management of patients with inherited primary arrhythmia syndromesHRS/EHRA/APHRS2013 (
      • Priori S.G.
      • Wilde A.A.
      • Horie M.
      • et al.
      2013 HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes.
      )
      Guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathyAHA/ACC2020 (
      • Ommen S.R.
      • Mital S.
      • Burke M.A.
      • 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.
      )
      ECS: The evaluation, risk stratification, and management of arrhythmogenic cardiomyopathyHRS2019 (
      • Towbin J.A.
      • McKenna W.J.
      • Abrams D.J.
      • et al.
      2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy.
      )
      Guidelines for the management for pediatric heart failureHRS2015 (
      • Kirk R.
      • Dipchand A.I.
      • Rosenthal D.N.
      • et al.
      2014 The International Society for Heart and Lung Transplantation guidelines for the management for pediatric heart failure.
      )
      ECS: CIED lead management and extractionHRS2017 (
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • Wilkoff B.L.
      • et al.
      2017 HRS expert consensus statement on cardiovascular implantable electronic device lead management and extraction.
      )
      ECS: MRI and radiation exposure in patients with CIEDsHRS2018 (
      • Indik J.H.
      • Gimbel J.R.
      • Abe H.
      • et al.
      2017 HRS expert consensus statement on magnetic resonance imaging and radiation exposure in patients with cardiovascular implantable electronic devices.
      )
      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).
      • Halperin J.L.
      • Levine G.N.
      • Al-Khatib S.M.
      • 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.
      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.
      • Kusumoto F.M.
      • 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.
      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 2Class of Recommendation and Level of Evidence Categories*

      1.2 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.

      1.3 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.

      1.4 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”
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      and “2012 ACCF/AHA/HRS Focused Update of the 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities.”
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      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.
      Tabled 1
      B-NR: Evidence from nonrandomized studies, observational studies, or registry studies
      C-LD: Very limited evidence from observational studies or case series reports
      C-EO: Consensus expert opinion, case studies, or standard of care
      *Adapted from Halperin, et al.
      • Halperin J.L.
      • Levine G.N.
      • Al-Khatib S.M.
      • 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.

      1.5 Top 10 Take-Home Messages

      • 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.
        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.
        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.
        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.
        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.
        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.
        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.
        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.
        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.
        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.

      2. Permanent Pacemakers

      2.1 Introduction

      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.
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      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.
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      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.
      • 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.
      ,
      • Khairy P.
      • Van Hare G.F.
      • Balaji S.
      • et al.
      2014 PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease.
      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.
      • Chang P.M.
      • Carter C.
      • Bar-Cohen Y.
      Indications for permanent pacing, device and lead selection.
      • Weindling S.
      • Saul J.
      • Triedman J.
      • et al.
      Staged pacing therapy for congenital complete heart block in premature infants.
      • Moore J.P.
      • Shannon K.M.
      Transpulmonary atrial pacing: an approach to transvenous pacemaker implantation after extracardiac conduit Fontan surgery.
      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.

      2.2 Isolated Sinus Node Dysfunction

      Tabled 1
      CORRecommendationsLOEReferences
      Isolated Sinus Node Dysfunction
      IPermanent atrial or dual-chamber pacemaker implantation is indicated for SND when there is correlation of symptoms with age-inappropriate bradycardia.B-NR
      • Breivik K.
      • Ohm O.J.
      • Segadal L.
      Sick sinus syndrome treated with permanent pacemaker in 109 patients: A follow-up study.
      • Albin G.
      • Hayes D.L.
      • Holmes Jr., D.R.
      Sinus node dysfunction in pediatric and young adult patients: Treatment by implantation of a permanent pacemaker in 39 cases.
      • Gillette P.C.
      • Shannon C.
      • Garson Jr., A.
      • et al.
      Pacemaker treatment of sick sinus syndrome in children.
      • Chiu S.N.
      • Lin L.Y.
      • Wang J.K.
      • et al.
      Long-term outcomes of pediatric sinus bradycardia.
      IPermanent pacemaker implantation is indicated in patients with symptomatic SND secondary to chronic medical therapy for which there is no alternative treatment.C-EO
      IIaPermanent pacemaker implantation (with rate-responsive programming) is reasonable in patients with symptoms temporally associated with observed chronotropic incompetence.C-LD
      • Reybrouck T.
      • Vangesselen S.
      • Gewillig M.
      Impaired chronotropic response to exercise in children with repaired cyanotic congenital heart disease.
      ,
      • 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.
      IIbPermanent pacemaker implantation may be considered in patients with SND and symptoms that are likely attributable to bradycardia or prolonged pauses without conclusive evidence correlating the symptoms with bradycardia following a thorough investigation.C-EO
      III

      No Benefit
      Permanent pacemaker implantation is not indicated in patients with asymptomatic SND.C-EO
      III

      Harm
      Permanent pacemaker implantation is not indicated in patients with symptomatic SND due to a reversible cause.C-EO
      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.
      • Breivik K.
      • Ohm O.J.
      • Segadal L.
      Sick sinus syndrome treated with permanent pacemaker in 109 patients: A follow-up study.
      • Albin G.
      • Hayes D.L.
      • Holmes Jr., D.R.
      Sinus node dysfunction in pediatric and young adult patients: Treatment by implantation of a permanent pacemaker in 39 cases.
      • Gillette P.C.
      • Shannon C.
      • Garson Jr., A.
      • et al.
      Pacemaker treatment of sick sinus syndrome in children.
      • Chiu S.N.
      • Lin L.Y.
      • Wang J.K.
      • et al.
      Long-term outcomes of pediatric sinus bradycardia.
      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.
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • 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.
      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.
      • Gillette P.C.
      • Wampler D.R.
      • Shannon C.
      • et al.
      Use of atrial pacing in a young population.

      2.3 Isolated Congenital Complete Atrioventricular Block

      Recommendation-Specific Supportive Text
      Tabled 1
      CORRecommendationsLOEReferences
      Isolated Congenital Complete Atrioventricular Block
      IPermanent pacemaker implantation is indicated for patients with CCAVB with symptomatic bradycardia.B-NR
      • Jaeggi E.T.
      • Hamilton R.M.
      • Silverman E.D.
      • et al.
      Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block.
      • Baruteau A.E.
      • 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.
      • 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.
      • Michaëlsson M.
      • Engle M.A.
      Isolated congenital complete atrioventricular block in adult life.
      IPermanent pacemaker implantation is indicated for patients with CCAVB with a wide QRS escape rhythm, complex ventricular ectopy, or ventricular dysfunction.B-NR
      • Michaëlsson M.
      • Engle M.A.
      Congenital complete heart block; an international study of the natural history.
      • Winkler R.B.
      • Freed M.D.
      • Nadas A.S.
      Exercise induced ventricular ectopy in children and young adults with complete heart block.
      • Karpawich P.P.
      • Gillette P.C.
      • Garson Jr., A.
      • et al.
      Congenital complete atrioventricular block: Clinical and electrophysiologic predictors of need for pacemaker insertion.
      IPermanent pacemaker implantation is indicated for CCAVB in asymptomatic neonates or infants when the mean ventricular rate is ≤50 bpm. Ventricular rate alone should not be used as implant criteria, as symptoms due to low cardiac output may occur at faster heart rates.C-LD
      • Jaeggi E.T.
      • Hamilton R.M.
      • Silverman E.D.
      • et al.
      Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block.
      ,
      • Michaëlsson M.
      • Engle M.A.
      Congenital complete heart block; an international study of the natural history.
      ,
      • Pinsky W.W.
      • Gillette P.C.
      • Garson A.
      • et al.
      Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block.
      IIaPermanent pacemaker implantation is reasonable for asymptomatic CCAVB beyond the first year of life when the mean ventricular rate is <50 bpm or there are prolonged pauses in ventricular rate.B-NR
      • Karpawich P.P.
      • Gillette P.C.
      • Garson Jr., A.
      • et al.
      Congenital complete atrioventricular block: Clinical and electrophysiologic predictors of need for pacemaker insertion.
      ,
      • Dewey R.C.
      • Capeless M.A.
      • Levy A.M.
      Use of ambulatory electrocardiographic monitoring to identify high-risk patients with congenital complete heart block.
      ,
      • Benson D.W.
      • Spach M.S.
      • Edwards S.B.
      • et al.
      Heart block in children. Evaluation of subsidiary ventricular pacemaker recovery times and ECG tape recordings.
      IIaPermanent pacemaker implantation is reasonable for CCAVB with left ventricular dilation (z score ≥3) associated with significant mitral insufficiency or systolic dysfunction.C-LD
      • Sholler G.F.
      • Walsh E.P.
      Congenital complete heart block in patients without anatomic cardiac defects.
      ,
      • Kertesz N.J.
      • Friedman R.A.
      • Colan S.D.
      • et al.
      Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block.
      IIbPermanent pacemaker implantation may be considered for CCAVB in asymptomatic adolescents with an acceptable ventricular rate, a narrow QRS complex, and normal ventricular function, based on an individualized consideration of the risk/benefit ratio.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Michaëlsson M.
      • Engle M.A.
      Isolated congenital complete atrioventricular block in adult life.
      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.
      • Glatz A.C.
      • Rhodes L.A.
      • Gayno J.W.
      • et al.
      Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth.
      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.
      • Michaëlsson M.
      • Engle M.A.
      Congenital complete heart block; an international study of the natural history.
      ,
      • Pinsky W.W.
      • Gillette P.C.
      • Garson A.
      • et al.
      Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block.
      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.
      • Jaeggi E.T.
      • Hamilton R.M.
      • Silverman E.D.
      • et al.
      Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block.
      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.
      • Michaëlsson M.
      • Engle M.A.
      Isolated congenital complete atrioventricular block in adult life.
      ,
      • Michaëlsson M.
      • Engle M.A.
      Congenital complete heart block; an international study of the natural history.
      ,
      • Moak J.P.
      • Barron K.S.
      • Hougen T.J.
      • et al.
      Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.
      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.
      • Moak J.P.
      • Barron K.S.
      • Hougen T.J.
      • et al.
      Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela.
      ,
      • Janoušek
      • van Geldorp I.E.
      • Krupičková S.
      • et al.
      Permanent cardiac pacing in children: Choosing the optimal pacing site: a multicenter study.

      2.4 Atrioventricular Block: Other Considerations

      Tabled 1
      CORRecommendationsLOEReferences
      Atrioventricular Block: Other Considerations
      IPermanent pacemaker implantation is indicated in patients with clinically significant VT that is pause dependent or associated with severe bradycardia; ICD implantation may be considered as a reasonable alternative.C-LD
      • Gladman G.
      • Davis A.M.
      • Fogelman R.
      • Hamilton R.M.
      • Gow R.M.
      Torsade de pointes, acquired complete heart block and inappropriately long QT in childhood.
      ,
      • Strasberg B.
      • Kusniec J.
      • Erdman S.
      • et al.
      Polymorphous ventricular tachycardia and atrioventricular block.
      IPermanent pacing is indicated in symptomatic patients with idiopathic advanced second- or third-degree AV block not attributable to reversible causes.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      IIaPermanent pacemaker implantation is reasonable for any degree of AV block that progresses to advanced second- or third-degree with exercise in the absence of reversible causes.C-LD
      • Yandrapalli S.
      • Harikrishnan P.
      • Ojo A.
      • Vuddanda V.L.K.
      • Jain D.
      Exercise induced complete atrioventricular block: Utility of exercise stress test.
      ,
      • Bonikowske A.R.
      • Barout A.
      • Fortin-Gamero S.
      • Lara M.I.B.
      • Kapa S.
      • Allison T.G.
      Frequency and characteristics of exercise-induced second-degree atrioventricular block in patients undergoing stress testing.
      IIbPermanent pacemaker implantation may be considered for patients with intermittent advanced second- or third-degree AV block not attributable to reversible causes and associated with minimal symptoms that are otherwise unexplained.C-LD
      • Silver E.S.
      • Pass R.H.
      • Hordof A.
      • Liberman
      Paroxysmal AV block in children with normal cardiac anatomy as a cause of syncope.
      III

      Harm
      Permanent pacemaker implantation is not indicated for asymptomatic first-degree AV block or asymptomatic second-degree Mobitz type I.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      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.
      • Silver E.S.
      • Pass R.H.
      • Hordof A.
      • Liberman
      Paroxysmal AV block in children with normal cardiac anatomy as a cause of syncope.
      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.
      • Yandrapalli S.
      • Harikrishnan P.
      • Ojo A.
      • Vuddanda V.L.K.
      • Jain D.
      Exercise induced complete atrioventricular block: Utility of exercise stress test.
      ,
      • Bonikowske A.R.
      • Barout A.
      • Fortin-Gamero S.
      • Lara M.I.B.
      • Kapa S.
      • Allison T.G.
      Frequency and characteristics of exercise-induced second-degree atrioventricular block in patients undergoing stress testing.
      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.

      2.5 Postoperative Atrioventricular Block

      Tabled 1
      CORRecommendationsLOEReferences
      Postoperative Atrioventricular Block
      IPermanent pacemaker implantation is indicated for postoperative advanced second- or third-degree AV block that persists for at least 7–10 days after cardiac surgery.B-NR
      • Weindling S.N.
      • Saul P.J.
      • Gamble W.J.
      • et al.
      Duration of complete atrioventricular block after congenital heart disease surgery.
      • Romer A.J.
      • Tabbutt S.
      • Etheridge S.P.
      • et al.
      Atrioventricular block after congenital heart surgery: Analysis from the Pediatric Cardiac Critical Care Consortium.
      • Aziz P.F.
      • Serwer G.A.
      • Bradley D.J.
      • 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.
      • Gross G.J.
      • Chiu C.C.
      • Hamilton R.M.
      • et al.
      Natural history of postoperative heart block in congenital heart disease: Implications for pacing intervention.
      IPermanent pacemaker implantation is indicated for late-onset advanced second- or third-degree AV block especially when there is a prior history of transient postoperative AV block.C-LD
      • Aziz P.F.
      • Serwer G.A.
      • Bradley D.J.
      • 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.
      ,
      • Krongrad E.
      Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects.
      ,
      • Villain E.
      • Ouarda F.
      • Beyler C.
      • et al.
      Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy.
      IIbPermanent pacemaker implantation may be considered for unexplained syncope in patients with a history of transient postoperative advanced second- or third-degree AV block.C-LD
      • Krongrad E.
      Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects.
      ,
      • Villain E.
      • Ouarda F.
      • Beyler C.
      • et al.
      Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy.
      IIbPermanent pacemaker implantation may be considered at <7 postoperative days when advanced second- or third-degree AV block is not expected to resolve due to extensive injury to the cardiac conduction system.C-EO
      IIbPermanent pacemaker implantation may be considered in select patients with transient postoperative advanced second- or third-degree AV block who are predisposed to progressive conduction abnormalities (see text).C-EO
      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.
      • Anderson J.B.
      • Czosek R.J.
      • Knilans T.K.
      • et al.
      Postoperative heart block in children with common forms of congenital heart disease: results from the KID Database.
      • Ayyildiz P.
      • Kasar T.
      • Ozturk E.
      • et al.
      Evaluation of permanent or transient complete heart block after open heart surgery for congenital heart disease.
      • Liberman L.
      • Pass R.H.
      • Hordof A.J.
      • et al.
      Incidence and characteristics of heart block after heart surgery in pediatric patients: A multicenter study.
      A very poor prognosis has been established for CHD patients with permanent postoperative AV block who do not receive permanent pacemakers.
      • Krongrad E.
      Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects.
      ,
      • Villain E.
      • Ouarda F.
      • Beyler C.
      • et al.
      Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy.
      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.
      • Weindling S.N.
      • Saul P.J.
      • Gamble W.J.
      • et al.
      Duration of complete atrioventricular block after congenital heart disease surgery.
      ,
      • Romer A.J.
      • Tabbutt S.
      • Etheridge S.P.
      • et al.
      Atrioventricular block after congenital heart surgery: Analysis from the Pediatric Cardiac Critical Care Consortium.
      Although patients who spontaneously regain AV conduction have a favorable prognosis,
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      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.
      • Aziz P.F.
      • Serwer G.A.
      • Bradley D.J.
      • 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.
      ,
      • Krongrad E.
      Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects.
      ,
      • Villain E.
      • Ouarda F.
      • Beyler C.
      • et al.
      Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy.
      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.
      • Krongrad E.
      Prognosis for patients with congenital heart disease and postoperative intraventricular conduction defects.
      ,
      • Villain E.
      • Ouarda F.
      • Beyler C.
      • et al.
      Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiomyopathy.
      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.
      • Huhta J.C.
      • Maloney J.D.
      • Ritter D.G.
      • et al.
      Complete atrioventricular block in patients with atrioventricular discordance.
      ,
      • Moore J.P.
      • Aboulhosn J.A.
      Introduction to the congenital heart defects: Anatomy of the conduction system.

      2.6 Congenital Heart Disease: Specific Considerations

      Tabled 1
      CORRecommendationsLOEReferences
      Congenital Heart Disease

      All the recommendations in children with a structurally normal heart apply, but in addition:
      IPermanent pacemaker implantation is indicated for CCAVB in neonates or infants with complex CHD when bradycardia is associated with hemodynamic compromise or when the mean ventricular rate is <60–70 bpm.C-LD
      • Glatz A.C.
      • Rhodes L.A.
      • Gayno J.W.
      • et al.
      Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth.
      ,
      • Jaeggi E.T.
      • Hornberger L.K.
      • Smallhorn J.F.
      • 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.
      ,
      • Lopes L.M.
      • Tavares G.M.
      • Damiano A.P.
      • et al.
      Perinatal outcome of fetal atrioventricular block: One-hundred-sixteen cases from a single institution.
      IIaPermanent pacemaker implantation with atrial antitachycardia pacing is reasonable for patients with CHD and recurrent episodes of intra-atrial re-entrant tachycardia when catheter ablation or medication are ineffective or not acceptable treatments.B-NR
      • Silka M.J.
      • Manwill J.R.
      • Kron J.
      • et al.
      Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates.
      • Rhodes L.A.
      • Walsh E.P.
      • Gamble W.J.
      • et al.
      Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease.
      • Kramer C.C.
      • Maldonado J.R.
      • Olson M.D.
      • et al.
      Safety and efficacy of atrial antitachycardia pacing in congenital heart disease.
      • Stephenson E.A.
      • Casavant D.
      • Tuzi J.
      • et al.
      Efficacy of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease.
      • Tsao S.
      • Deal B.J.
      • Backer C.L.
      • et al.
      Device management of arrhythmias after Fontan conversion.
      IIaPermanent atrial or dual-chamber pacemaker implantation is reasonable for patients with CHD and impaired hemodynamics due to sinus bradycardia or loss of AV synchrony.C-LD
      • Silka M.J.
      • Manwill J.R.
      • Kron J.
      • et al.
      Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates.
      ,
      • Barber B.J.
      • Batra A.S.
      • Burch G.H.
      • et al.
      Acute hemodynamic effects of pacing in patients with Fontan physiology: A prospective study.
      IIaPermanent atrial or dual-chamber pacing is reasonable for patients with tachy-brady syndrome and symptoms attributable to pauses due to sudden-onset bradycardia.C-LD
      • Silka M.J.
      • Manwill J.R.
      • Kron J.
      • et al.
      Bradycardia-mediated tachyarrhythmias in congenital heart disease and responses to chronic pacing at physiologic rates.
      ,
      • Drago F.
      • Silvetti M.S.
      • Grutter G.
      • et al.
      Use of DDDRP pacing device in prevention and treatment of tachy-brady syndrome after Mustard procedure.
      IIaPermanent pacemaker implantation is reasonable for sinus or junctional bradycardia with complex CHD
      • Stout K.K.
      • Daniels C.J.
      • Aboulhosn
      • 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.
      when the mean awake resting heart rate is <40 bpm or when there are prolonged pauses in the ventricular rate.
      C-EO
      IIbPermanent pacing may be considered for sinus or junctional bradycardia with simple or moderate CHD
      • Stout K.K.
      • Daniels C.J.
      • Aboulhosn
      • 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.
      when the mean awake resting heart rate is <40 bpm or when there are prolonged pauses in the ventricular rate.
      C-EO
      III

      Harm
      Endocardial leads should be avoided in patients with CHD and intracardiac shunt except in select cases, for whom there should be an individualized consideration of the risk/benefit ratio. In these exceptional cases anticoagulation is mandatory, but thromboembolism remains a risk.B-NR
      • Khairy P.
      • Landzberg M.
      • Gatzoulis M.A.
      • 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.
      • DeSimone C.V.
      • Friedman P.A.
      • Noheria A.
      • et al.
      Stroke or transient ischemic attack in patients with transvenous pacemaker or defibrillator and echocardiographically detected patent foramen ovale.
      • Supple G.E.
      • Ren J.-F.
      • Zado E.S.
      • Marchlinski F.E.
      Mobile thrombus on device leads in patients undergoing ablation.
      Recommendation-Specific Supportive Text
      Patients with CHD often have important structural and functional lesions,
      • Stout K.K.
      • Daniels C.J.
      • Aboulhosn
      • 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.
      which influence both the indications for pacing as well as the type of pacing lead(s) utilized.
      • Glatz A.C.
      • Rhodes L.A.
      • Gayno J.W.
      • et al.
      Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth.
      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),
      • Lau K.C.
      • Gaynor J.W.
      • Fuller S.M.
      • et al.
      Long term atrial and ventricular epicardial pacemaker lead survival after cardiac operations in pediatric patients with congenital heart disease.
      although novel hybrid approaches to lead placement are being developed.
      • Termosesov S.
      • Kulbachinskaya E.
      • Polyakava E.
      • et al.
      Video-assisted thoracoscopic pacemaker lead placement in children with atrioventricular block.
      ,
      • 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.
      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.
      • Tsao S.
      • Deal B.J.
      • Backer C.L.
      • et al.
      Device management of arrhythmias after Fontan conversion.
      ,
      • Barber B.J.
      • Batra A.S.
      • Burch G.H.
      • et al.
      Acute hemodynamic effects of pacing in patients with Fontan physiology: A prospective study.
      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.
      • Huhta J.C.
      • Maloney J.D.
      • Ritter D.G.
      • et al.
      Complete atrioventricular block in patients with atrioventricular discordance.
      ,
      • Moore J.P.
      • Aboulhosn J.A.
      Introduction to the congenital heart defects: Anatomy of the conduction system.
      ,
      • Cohen M.I.
      • Rhodes L.A.
      • Spray T.L.
      Efficacy of prophylactic epicardial pacing leads in children and young adults.
      Similarly, in single-ventricle patients undergoing Fontan conversion, prophylactic antitachycardia pacemakers have been used.
      • Tsao S.
      • Deal B.J.
      • Backer C.L.
      • et al.
      Device management of arrhythmias after Fontan conversion.
      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.
      • Rychik J.
      • Atz A.M.
      • Celermajer D.S.
      • Deal B.J.
      • et al.
      Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association.
      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.
      • Stout K.K.
      • Daniels C.J.
      • Aboulhosn
      • 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.

      2.7 Post Cardiac Transplantation

      Tabled 1
      CORRecommendationsLOEReferences
      Post Cardiac Transplantation
      IPermanent pacing is indicated for persistent symptomatic bradycardia that is not expected to resolve and for other class I indications for permanent pacing.C-LD
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Kertesz N.J.
      • Towbin J.A.
      • Clunie S.
      • et al.
      Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.
      • El-Assaad I.
      • Al-Kindi S.G.
      • Oliveira G.H.
      • et al.
      Pacemaker implantation in pediatric heart transplant recipients: Predictors, outcomes, and impact on survival.
      • Jones D.G.
      • Mortsell D.H.
      • Rajaruthnam D.
      • et al.
      Permanent pacemaker implantation early and late after heart transplantation: clinical indication, risk factors and prognostic implications.
      • Mahmood A.
      • Andrews R.
      • Fenton M.
      • et al.
      Permanent pacemaker implantation after pediatric heart transplantation: Risk factors, indications, and outcomes.
      IIaPermanent pacing is reasonable for marked chronotropic incompetence impairing the quality of life late in the post-transplant period.C-LD
      • Kertesz N.J.
      • Towbin J.A.
      • Clunie S.
      • et al.
      Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.
      • El-Assaad I.
      • Al-Kindi S.G.
      • Oliveira G.H.
      • et al.
      Pacemaker implantation in pediatric heart transplant recipients: Predictors, outcomes, and impact on survival.
      • Jones D.G.
      • Mortsell D.H.
      • Rajaruthnam D.
      • et al.
      Permanent pacemaker implantation early and late after heart transplantation: clinical indication, risk factors and prognostic implications.
      • Mahmood A.
      • Andrews R.
      • Fenton M.
      • et al.
      Permanent pacemaker implantation after pediatric heart transplantation: Risk factors, indications, and outcomes.
      IIbPermanent pacing may be considered when relative bradycardia is prolonged, recurrent, or limits rehabilitation or discharge after postoperative recovery from cardiac transplantation.C-LD
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Jones D.G.
      • Mortsell D.H.
      • Rajaruthnam D.
      • et al.
      Permanent pacemaker implantation early and late after heart transplantation: clinical indication, risk factors and prognostic implications.
      ,
      • Luebbert J.J.
      • Lee F.A.
      • Rosenfeld L.E.
      Pacemaker therapy for early and late sinus node dysfunction in orthotopic heart transplant recipients: a single-center experience.
      IIbPermanent pacing may be considered for any degree of AV block considered to be due to graft vasculopathy.C-LD
      • Kertesz N.J.
      • Towbin J.A.
      • Clunie S.
      • et al.
      Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.
      ,
      • Cannon B.C.
      • Denfeld S.W.
      • Friedman R.A.
      • et al.
      Late pacemaker requirement after pediatric orthotopic heart transplantation may predict the presence of transplant coronary artery disease.
      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.
      • Kertesz N.J.
      • Towbin J.A.
      • Clunie S.
      • et al.
      Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.
      ,
      • El-Assaad I.
      • Al-Kindi S.G.
      • Oliveira G.H.
      • et al.
      Pacemaker implantation in pediatric heart transplant recipients: Predictors, outcomes, and impact on survival.
      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.
      • El-Assaad I.
      • Al-Kindi S.G.
      • Oliveira G.H.
      • et al.
      Pacemaker implantation in pediatric heart transplant recipients: Predictors, outcomes, and impact on survival.
      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.
      • Kertesz N.J.
      • Towbin J.A.
      • Clunie S.
      • et al.
      Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.
      ,
      • Cannon B.C.
      • Denfeld S.W.
      • Friedman R.A.
      • et al.
      Late pacemaker requirement after pediatric orthotopic heart transplantation may predict the presence of transplant coronary artery disease.
      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.
      • Chang A.C.
      • Hruban R.H.
      • Levin H.R.
      • et al.
      Comparison of rejection in the atrioventricular node and bundles with the working myocardium in transplanted hearts.
      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.
      • Daly K.P.
      • Chakravarti S.B.
      • Tresler M.
      • et al.
      Sudden death after pediatric heart transplantation: Analysis of data from the Pediatric Heart Transplant Study Group.
      ,
      • Carboni M.P.
      Sudden cardiac death after heart transplantation: Can ICD prevent SCD?.

      2.8 Neuromuscular Diseases and Other Progressive Cardiac Conduction Diseases

      Tabled 1
      CORRecommendationsLOEReferences
      Neuromuscular Diseases and Other Progressive Cardiac Conduction Diseases
      IPermanent pacemaker implantation is indicated in patients with neuromuscular diseases with symptomatic bradycardia due to SND or any degree of AV block.B-NR
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Feingold B.
      • Mahle W.T.
      • Auerbach S.
      • et al.
      Management of cardiac involvement associated with neuromuscular diseases: A scientific statement from the American Heart Association.
      • Bhakta D.
      • Shen C.
      • Kron J.
      • et al.
      Pacemaker and implantable cardioverter-defibrillator use in a us myotonic dystrophy type 1 population.
      • Lund M.
      • Diaz K.J.
      • Ranthe M.F.
      • et al.
      Cardiac involvement in myotonic dystrophy: A nationwide cohort study.
      • Ha A.H.
      • Tarnopolsky M.A.
      • Bergstra T.G.
      • et al.
      Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II.
      • Groh W.J.
      • Groh M.R.
      • Saha C.
      • et al.
      Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.
      • Wahbi K.
      • Meune C.
      • Porcher R.
      • et al.
      Electrophysiological study with prophylactic pacing and survival in adults with myotonic dystrophy and conduction system disease.
      • Van Berlo J.H.
      • de Voogt W.G.
      • van der Kooi A.J.
      • 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?.
      • Polak P.E.
      • Zijlstra F.
      • Roelandt J.R.
      Indications for pacemaker implantation in the Kearns-Sayre syndrome.
      IPermanent pacemaker implantation is indicated in Kearns-Sayre syndrome for any degree of AV block (including first-degree AV block) and/or conduction abnormality because of unpredictable progression of conduction disease.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Polak P.E.
      • Zijlstra F.
      • Roelandt J.R.
      Indications for pacemaker implantation in the Kearns-Sayre syndrome.
      • Kabunga P.
      • Lau A.K.
      • Phan K.
      • et al.
      Systematic review of cardiac electrical disease in Kearns-Sayre syndrome and mitochondrial cytopathy.
      • Khambatta S.
      • Nguyen D.L.
      • Beckman T.J.
      • et al.
      Kearns-Sayre syndrome: A case series of 35 adults and children.
      • Di Mambro C.
      • Tamborrino P.P.
      • Silvetti M.S.
      • 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?.
      IIaPermanent pacemaker implantation is reasonable in patients with myotonic dystrophy type 1 for marked first-degree AV block (PR interval >240 ms) or intraventricular conduction delay (native QRS duration >120 ms). Additional defibrillator capability may be considered.B-NR
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Feingold B.
      • Mahle W.T.
      • Auerbach S.
      • et al.
      Management of cardiac involvement associated with neuromuscular diseases: A scientific statement from the American Heart Association.
      ,
      • Lund M.
      • Diaz K.J.
      • Ranthe M.F.
      • et al.
      Cardiac involvement in myotonic dystrophy: A nationwide cohort study.
      ,
      • Ha A.H.
      • Tarnopolsky M.A.
      • Bergstra T.G.
      • et al.
      Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II.
      IIaPermanent pacemaker implantation is reasonable in patients with lamin A/C gene mutations, including limb-girdle and Emery-Dreifuss muscular dystrophies with a PR interval >240 ms and/or left bundle branch block. Additional defibrillator capability may be considered.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Van Berlo J.H.
      • de Voogt W.G.
      • van der Kooi A.J.
      • 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?.
      ,
      • Hasselberg N.E.
      • Edvardsen T.
      • Petri H.
      • Berge K.E.
      • et al.
      Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects.
      IIbPermanent pacemaker implantation may be considered for any patient with any progressive cardiac conduction disease with potential for rapid deterioration of AV nodal function, even in the presence of normal AV conduction after taking into consideration patient age, size, and other individual risk factors.C-LD
      • Kusumoto F.M.
      • Schoenfeld M.H.
      • 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.
      ,
      • Feingold B.
      • Mahle W.T.
      • Auerbach S.
      • et al.
      Management of cardiac involvement associated with neuromuscular diseases: A scientific statement from the American Heart Association.
      ,
      • Lund M.
      • Diaz K.J.
      • Ranthe M.F.
      • et al.
      Cardiac involvement in myotonic dystrophy: A nationwide cohort study.
      ,
      • Ha A.H.
      • Tarnopolsky M.A.
      • Bergstra T.G.
      • et al.
      Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II.
      ,
      • Asatryan B.
      • Medeiros-Domingo A.
      Molecular and genetic insights into progressive cardiac conduction disease.
      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.
      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.
      • Jones D.G.
      • Mortsell D.H.
      • Rajaruthnam D.
      • et al.
      Permanent pacemaker implantation early and late after heart transplantation: clinical indication, risk factors and prognostic implications.
      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.
      • Carboni M.P.
      Sudden cardiac death after heart transplantation: Can ICD prevent SCD?.
      ,
      • Groh W.J.
      • Groh M.R.
      • Saha C.
      • et al.
      Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.
      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.
      • Carboni M.P.
      Sudden cardiac death after heart transplantation: Can ICD prevent SCD?.
      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.
      • Ha A.H.
      • Tarnopolsky M.A.
      • Bergstra T.G.
      • et al.
      Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II.
      ,
      • Groh W.J.
      • Groh M.R.
      • Saha C.
      • et al.
      Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.
      ,
      • Van Berlo J.H.
      • de Voogt W.G.
      • van der Kooi A.J.
      • 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?.
      ,
      • Hasselberg N.E.
      • Edvardsen T.
      • Petri H.
      • Berge K.E.
      • et al.
      Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects.
      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.
      • Feingold B.
      • Mahle W.T.
      • Auerbach S.
      • et al.
      Management of cardiac involvement associated with neuromuscular diseases: A scientific statement from the American Heart Association.
      • Bhakta D.
      • Shen C.
      • Kron J.
      • et al.
      Pacemaker and implantable cardioverter-defibrillator use in a us myotonic dystrophy type 1 population.
      • Lund M.
      • Diaz K.J.
      • Ranthe M.F.
      • et al.
      Cardiac involvement in myotonic dystrophy: A nationwide cohort study.
      • Ha A.H.
      • Tarnopolsky M.A.
      • Bergstra T.G.
      • et al.
      Predictors of atrio-ventricular conduction disease, long-term outcomes in patients with myotonic dystrophy types I and II.
      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.

      2.9 Neurocardiogenic Syncope

      Tabled 1
      CORRecommendationsLOEReferences
      Neurocardiogenic Syncope
      IIaPermanent pacemaker implantation is reasonable with severe recurrent breath-holding spells with documentation of cardioinhibitory response on ECG monitoring and complicated by prolonged syncope, prolonged postanoxic convulsions, and other bradycardia-induced symptoms.B-NR
      • Kolterer B.
      • Gebauer R.A.
      • Janousek J.
      • Dähnert I.
      • Riede F.T.
      • Paech C.
      Improved quality of life after treatment of prolonged asystole during breath holding spells with a cardiac pacemaker.
      • McLeod K.A.
      • Wilson N.
      • Hewitt J.
      • Norrie J.
      • Stephenson J.B.
      Cardiac pacing for severe childhood neurally mediated syncope with reflex anoxic seizures.
      • Kelly A.M.
      • Porter C.J.
      • McGoon M.D.
      • Espinosa R.E.
      • Osborn M.J.
      • Hayes D.L.
      Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation.
      IIbPermanent pacing may be considered for recurrent symptomatic neurocardiogenic syncope associated with documented spontaneous bradycardia or asystole in patients who have failed other medical treatments.C-LD
      • Brignole M.
      • Menozzi C.
      • Moya A.
      • et al.
      International Study on Syncope of Uncertain Etiology 3 (ISSUE-3) Investigators. Pacemaker therapy in patients with neurally mediated syncope and documented asystole: Third International Study on Syncope of Uncertain Etiology (ISSUE-3): a randomized trial.
      • Paech C.
      • Wagner F.
      • Mensch S.
      • Antonin Gebauer R.
      Cardiac pacing in cardioinhibitory syncope in children.
      • Sutton R.
      • de Jong JSY
      • Stewart J.M.
      • et al.
      Pacing in vasovagal syncope: Physiology, pacemaker sensors, and recent clinical trials-Precise patient selection and measurable benefit.
      IIbPermanent pacemaker implantation may be considered in patients with epilepsy associated with severe symptomatic bradycardia (ictal induced) who have failed to improve with antiepileptic medical therapy.C-LD
      • Benditt D.G.
      • van Dijk G.
      • Thijs R.D.
      Ictal asystole: life-threatening vagal storm or a benign seizure self-termination mechanism?.
      ,
      • Bestawros M.
      • Darbar D.
      • Arain A.
      • Abou-Khalil B.
      • Plummer D.
      • Dupont W.D.
      • Rah S.R.
      Ictal Asystole and Ictal Syncope: Insights into Clinical Management.
      III

      No benefit
      Permanent pacing is not indicated for neurocardiogenic syncope solely on the basis of a positive cardioinhibitory tilt response.C-EO
      III

      Harm
      Permanent pacing is not indicated for neurocardiogenic syncope with hypotension as the major or significant component of the symptoms.C-EO
      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.
      • Kolterer B.
      • Gebauer R.A.
      • Janousek J.
      • Dähnert I.
      • Riede F.T.
      • Paech C.
      Improved quality of life after treatment of prolonged asystole during breath holding spells with a cardiac pacemaker.
      • McLeod K.A.
      • Wilson N.
      • Hewitt J.
      • Norrie J.
      • Stephenson J.B.
      Cardiac pacing for severe childhood neurally mediated syncope with reflex anoxic seizures.
      • Kelly A.M.
      • Porter C.J.
      • McGoon M.D.
      • Espinosa R.E.
      • Osborn M.J.
      • Hayes D.L.
      Breath-holding spells associated with significant bradycardia: successful treatment with permanent pacemaker implantation.
      • Brignole M.
      • Menozzi C.
      • Moya A.
      • et al.
      International Study on Syncope of Uncertain Etiology 3 (ISSUE-3) Investigators. Pacemaker therapy in patients with neurally mediated syncope and documented asystole: Third International Study on Syncope of Uncertain Etiology (ISSUE-3): a randomized trial.
      ,
      • Bestawros M.
      • Darbar D.
      • Arain A.
      • Abou-Khalil B.
      • Plummer D.
      • Dupont W.D.
      • Rah S.R.
      Ictal Asystole and Ictal Syncope: Insights into Clinical Management.
      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.
      • Paech C.
      • Wagner F.
      • Mensch S.
      • Antonin Gebauer R.
      Cardiac pacing in cardioinhibitory syncope in children.
      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.
      • Kolterer B.
      • Gebauer R.A.
      • Janousek J.
      • Dähnert I.
      • Riede F.T.
      • Paech C.
      Improved quality of life after treatment of prolonged asystole during breath holding spells with a cardiac pacemaker.
      ,
      • McLeod K.A.
      • Wilson N.
      • Hewitt J.
      • Norrie J.
      • Stephenson J.B.
      Cardiac pacing for severe childhood neurally mediated syncope with reflex anoxic seizures.
      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.
      • Sutton R.
      • de Jong JSY
      • Stewart J.M.
      • et al.
      Pacing in vasovagal syncope: Physiology, pacemaker sensors, and recent clinical trials-Precise patient selection and measurable benefit.
      ,
      • Benditt D.G.
      • van Dijk G.
      • Thijs R.D.
      Ictal asystole: life-threatening vagal storm or a benign seizure self-termination mechanism?.
      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.

      2.10 Cardiac Channelopathies

      Tabled 1
      CORRecommendationsLOEReferences
      Cardiac Channelopathies
      IPermanent pacemaker implantation is indicated in channelopathy patients with pause-dependent, clinically significant VT; ICD implantation may be considered as a reasonable alternative.C-LD
      • Moss A.J.
      • Liu J.E.
      • Gottlieb S.
      • Locati E.H.
      • et al.
      Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome.
      • Eldar M.
      • Griffin J.C.
      • Van Hare G.F.
      • et al.
      Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome.
      • Viskin S.
      • Fish R.
      • Zeltser D.
      • et al.
      Arrhythmias in the congenital long QT syndrome: How often is torsade de pointes pause dependent?.
      IIbPermanent pacemaker implantation may be considered as adjunctive therapy in patients with long QT syndrome and functional 2:1 AV block.C-LD
      • Aziz P.F.
      • Tanel R.E.
      • Zelster I.J.
      • et al.
      Congenital long QT syndrome and 2:1 atrioventricular block: an optimistic outcome in the current era.
      IIbPermanent pacemaker implantation may be considered as adjunctive therapy in patients with long QT syndrome or other channelopathies where a faster heart rate may decrease the arrhythmia burden or symptoms due to bradycardia.C-LD
      • Moss A.J.
      • Liu J.E.
      • Gottlieb S.
      • Locati E.H.
      • et al.
      Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome.
      ,
      • Eldar M.
      • Griffin J.C.
      • Abbott J.A.
      • et al.
      Permanent cardiac pacing in patients with the long QT syndrome.
      ,
      • Kowlgi G.N.
      • Giudicessi J.R.
      • Brake W.
      • et al.
      Efficacy of intentional permanent atrial pacing in the long-term management of congenital long QT syndrome.
      III

      No benefit
      Atrial pacing alone is not indicated in patients with complete atrial standstill due to the high potential for noncapture of the myocardium.C-LD
      • 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.
      ,
      • Ishikawa T.
      • Tsuji Y.
      • Makita N.
      Inherited bradyarrhythmia: A diverse genetic background.
      Recommendation-Specific Supportive text
      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.
      • Moss A.J.
      • Liu J.E.
      • Gottlieb S.
      • Locati E.H.
      • et al.
      Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome.
      • Eldar M.
      • Griffin J.C.
      • Van Hare G.F.
      • et al.
      Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome.
      • Viskin S.
      • Fish R.
      • Zeltser D.
      • et al.
      Arrhythmias in the congenital long QT syndrome: How often is torsade de pointes pause dependent?.
      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.
      • Aziz P.F.
      • Tanel R.E.
      • Zelster I.J.
      • et al.
      Congenital long QT syndrome and 2:1 atrioventricular block: an optimistic outcome in the current era.
      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.
      • Moss A.J.
      • Liu J.E.
      • Gottlieb S.
      • Locati E.H.
      • et al.
      Efficacy of permanent pacing in the management of high-risk patients with long QT syndrome.
      ,
      • Kowlgi G.N.
      • Giudicessi J.R.
      • Brake W.
      • et al.
      Efficacy of intentional permanent atrial pacing in the long-term management of congenital long QT syndrome.
      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.
      • 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.
      ,
      • Ishikawa T.
      • Tsuji Y.
      • Makita N.
      Inherited bradyarrhythmia: A diverse genetic background.

      2.11 Inflammation/Infection

      Tabled 1
      CORRecommendationsLOEReferences
      Inflammation/Infection
      IPermanent pacing is indicated in patients with high-grade or symptomatic AV block attributable to a known potentially reversible cause when AV block does not resolve despite treatment of the underlying cause.C-LD
      • McAlister H.F.
      • Klementowicz P.T.
      • Andrews C.
      • et al.
      Lyme carditis: an important cause of reversible heart block.
      ,
      • Forrester J.D.
      • Mead P.
      Third-degree heart block associated with lyme carditis: review of published cases.
      IIaPacemaker implantation is reasonable in Chagas disease and advanced second- or third-degree AV block, as spontaneous resolution is unlikely. ICD implantation may be a reasonable alternative.C-LD
      • McAlister H.F.
      • Klementowicz P.T.
      • Andrews C.
      • et al.
      Lyme carditis: an important cause of reversible heart block.
      • Forrester J.D.
      • Mead P.
      Third-degree heart block associated with lyme carditis: review of published cases.
      • Nunes M.C.P.
      • Beaton A.
      • Acquatella H.
      • et al.
      Chagas Cardiomyopathy: An Update of Current Clinical Knowledge and Management: A Scientific Statement from the American Heart Association.
      • Bocchi E.A.
      • Bestetti R.B.
      • Scanavacca M.I.
      • et al.
      Chronic Chagas Heart Disease Management: From Etiology to Cardiomyopathy Treatment.
      III

      No benefit
      Permanent pacing should not be performed in patients who had acute AV block attributable to a known reversible cause, when there is recovery of normal AV conduction.C-EO
      Recommendation-Specific Supportive Text
      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.
      • McAlister H.F.
      • Klementowicz P.T.
      • Andrews C.
      • et al.
      Lyme carditis: an important cause of reversible heart block.
      ,
      • Forrester J.D.
      • Mead P.
      Third-degree heart block associated with lyme carditis: review of published cases.
      Chronic Chagas disease can present with different degrees of conduction defects. Advanced heart block in Chagas is permanent, and pacemaker implantation is indicated.
      • Nunes M.C.P.
      • Beaton A.
      • Acquatella H.
      • et al.
      Chagas Cardiomyopathy: An Update of Current Clinical Knowledge and Management: A Scientific Statement from the American Heart Association.
      ,
      • Bocchi E.A.
      • Bestetti R.B.
      • Scanavacca M.I.
      • et al.
      Chronic Chagas Heart Disease Management: From Etiology to Cardiomyopathy Treatment.
      An ICD should be considered in Chagas cardiomyopathy in the presence of significant left ventricular dysfunction or ventricular arrhythmias.
      • Bocchi E.A.
      • Bestetti R.B.
      • Scanavacca M.I.
      • et al.
      Chronic Chagas Heart Disease Management: From Etiology to Cardiomyopathy Treatment.
      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.
      • Dionne A.
      • Mah D.
      • Son M.F.
      • et al.
      Atrio-Ventricular Block in Children With Multisystem Inflammatory Syndrome.
      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.
      • Batra A.S.
      • Epstein D.
      • Silka M.J.
      The clinical course of acquired complete heart block in children with acute myocarditis.
      Late monitoring for possible recurrence of symptoms or unrecognized recurrences of AV block or other arrhythmias is advised in these patients.

      3. Implantable Cardioverter Defibrillators

      3.1 Introduction

      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.
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Al-Khatib S.M.
      • Stevenson W.G.
      • Ackerman M.J.
      • et al.
      2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death.
      ,
      • Priori S.G.
      • 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.
      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.
      • Cunningham T.
      • Roston T.M.
      • Franciosi S.
      • et al.
      Initially unexplained cardiac arrest in children and adolescents: a national experience from the Canadian Pediatric Heart Rhythm Network.
      ,
      • Rucinski C.
      • Winbo A.
      • Marcondes L.
      • et al.
      A Population-Based Registry of Patients with Inherited Cardiac Conditions and Resuscitated Cardiac Arrest.
      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.
      • Silka M.J.
      • Kobayashi R.L.
      • Hill A.C.
      • et al.
      Pediatric survivors of out-of-hospital ventricular fibrillation: etiologies and outcomes.
      ,
      • van der Werf C.
      • Lieve K.V.
      • Bos J.M.
      • et al.
      Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest.
      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.
      • Cohen M.I.
      • Etheridge S.P.
      Indications for implantable cardioverter defibrillator therapy, device and lead selection.
      ,
      • Minier M.
      • Probst V.
      • Berthome P.
      • et al.
      Age at diagnosis of Brugada syndrome: Influence on clinical characteristics and risk of arrhythmia.
      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.

      3.2 General Recommendations for Implantable Cardioverter Defibrillator Therapy

      Tabled 1
      CORRecommendationsLOEReferences
      General Recommendations for Implantable Cardioverter Defibrillator Therapy
      IICD implantation is indicated for survivors of SCA due to VT/VF if completely reversible causes have been excluded and an ICD is considered to be more beneficial than alternative treatments that may significantly reduce the risk of SCA.B-NR
      • Epstein A.E.
      • Dimarco J.P.
      • Ellenbogen K.A.
      • et al.
      ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Tracy C.M.
      • Epstein A.E.
      • Darbar D.
      • et al.
      2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities.
      ,
      • Al-Khatib S.M.
      • Stevenson W.G.
      • Ackerman M.J.
      • et al.
      2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death.
      ,
      • Priori S.G.
      • 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.
      ,
      • Silka M.J.
      • Kron J.
      • Dunnigan A.
      • Dick 2nd, M.
      Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients. The Pediatric Electrophysiology Society.
      • Priori S.G.
      • 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.
      • Berul C.I.
      • Van Hare G.F.
      • Kertesz N.J.
      • et al.
      Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients.
      • Von Bergen N.H.
      • Atkins D.L.
      • Dick 2nd, M.
      • et al.
      Multicenter study of the effectiveness of implantable cardioverter defibrillators in children and young adults with heart disease.
      • Baskar S.
      • Bao H.
      • Minges K.E.
      • Spar D.S.
      • et al.
      Characteristics and Outcomes of Pediatric Patients Who Undergo Placement of Implantable Cardioverter Defibrillators: Insights From the National Cardiovascular Data Registry.
      IIbICD implantation may be considered for patients with sustained VT that cannot be adequately controlled with medication and/or catheter ablation.C-EO
      IIbICD therapy may be considered for primary prevention of SCD in patients with genetic cardiovascular diseases and risk factors for SCA or pathogenic mutations and family history of recurrent SCA.C-EO
      III

      Harm
      ICD therapy is not indicated for patients with incessant ventricular tachyarrhythmias due to risk of ICD storm.C-EO
      III

      Harm
      ICD therapy is not indicated for patients with ventricular arrhythmias that are adequately treated with medication and/or catheter ablation.C-LD
      • Collins K.K.
      • Schaffer M.S.
      • Liberman L.
      • et al.
      Fascicular and nonfascicular left ventricular tachycardias in the young: an international multicenter study.
      • Roggen A.
      • Pavlovic M.
      • Pfammatter J.P.
      Frequency of spontaneous ventricular tachycardia in a pediatric population.