2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients (2022)

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; LOE, level of evidence; LQTS, long QT syndrome; LVEF, left ventricular ejection fraction; LVNC, left ventricular noncompaction; MRI, magnetic resonance imaging; NIDCM, nonischemic dilated cardiomyopathy; RIM, remote interrogation and monitoring; SND, sinus node dysfunction; SCA, sudden cardiac arrest; SCD, sudden cardiac death; VF, ventricular fibrillation; VT, ventricular tachycardia This article has been copublished in Heart Rhythm, Indian Pacing and Electrophysiology Journal, Cardiology in the Young, and Annals of Pediatric Cardiology. The Pediatric Cardiac Society requests that this document be cited as follows: Silka MJ, Shah MJ, Silva JNA, Balaji S, Beach CM, Benjamin MN, Berul CI, Cannon B, Cecchin F, Cohen MI, Dalal AS, Dechert BE, Foster A, Gebauer R, Gonzalez Corcia MC, Kannankeril PJ, Karpawich PP, Kim JJ, Krishna MR, Kubuš P, LaPage MJ, Mah DY, Malloy-Walton L, Miyazaki A, Motonaga KS, Niu MC, Olen M, Paul T, Rosenthal E, Saarel EV, Silvetti MS, Stephenson EA, Tan RB, Triedman J, Von Bergen NH, Wackel PL. 2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary. Annals of Pediatric Cardiology 2021:18;1-26. Address reprint requests and correspondence: Dr Michael J. Silka, Division of Pediatric Cardiology, Children's Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, CA 90027. E-mail address: [email protected]; or Dr Maully J. Shah, Cardiac Center, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104. E-mail address: [email protected]TABLE: OF CONTENTSPREAMBLE Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984.1 CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay,2 which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document,3 with further data easily accessible in electronic searches or textbooks. 1 INTRODUCTION 1.1 Methodology and Evidence Review The principles in the development of this document are 1) new recommendations or changes to previous recommendations are based on data, when possible; 2) these recommendations are consistent with current ACC/AHA/HRS adult guidelines when reasonable; and 3) all recommendations have been critically reviewed, initially by the writing committee and editors, followed by the PACES executive committee, and subsequently by external HRS, ACCF, AHA, and AEPC representatives. Any revisions or additions to existing recommendations require approval of at least 80% by the members of the PACES writing committee. These recommendations have been developed with standard guideline methodology, i.e., with both a class of recommendation (COR) and a level of evidence (LOE) Table 1. 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 evidence based on the type, quantity, and consistency of data from clinical trials and other sources. 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. However, there may be unequivocal consensus that a particular intervention is either effective or necessary.Table 1: Class 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”5 and “2012 ACCF/AHA/HRS Focused Update of the 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities.”6 1.5 Top 10 Take-Home Messages 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. 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. 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. 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. Atrial pacing with antitachycardia pacing capabilities is reasonable for congenital heart disease (CHD) patients with recurrent intra-atrial reentrant tachycardia when medication and catheter ablation are not effective. 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. 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. 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. 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. 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 are 1) symptomatic sinus bradycardia, 2) advanced second- or third-degree AV block, and 3) pacing for the prevention or termination of tachyarrhythmias.5 Many indications for pacemaker implantation in adolescents are similar to those in adults.2 However, in infants and young children, there are important differences. For example, criteria for normal heart rates are an age-dependent variable; whereas a heart rate of 45 bpm is 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.[78] 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 lead placement and innovative use of device technology may be needed to provide pacing or defibrillation in young patients. Furthermore, as device leads may need to be utilized for multiple decades, consideration of the from lead a in implantation of pediatric node dysfunction to either due to bradycardia or in the cardiac In patients with isolated sinus bradycardia symptoms due to or there is no minimum heart rate or maximum pause duration where permanent pacing is Establishing a temporal correlation between symptoms and bradycardia is of when whether permanent pacing is In symptomatic patients with SND, pacing is recommended ventricular The average ventricular rate in and infants with isolated provides objective regarding the decision for pacemaker implantation. However, additional factors including birth weight (size), ventricular and other may equally influence the Therefore, an average heart rate of bpm is recommended for infant pacemaker implantation when symptoms to cardiac are not the of permanent pacemaker implantation is in symptomatic patients. have dysfunction and with cardiovascular in the or in patients who not pacemaker AV block or may be to or remain times, AV block may be and to document. may be useful regarding the of AV AV block conduction the is and is with a the of or the criteria for pacemaker implantation are similar to those for supportive text AV block of congenital heart with of patients permanent pacemaker implantation for postoperative AV A has been established for CHD patients with permanent postoperative AV block who do not permanent patients who AV conduction following AV block, have of AV conduction by and AV conduction by postoperative Although patients who AV conduction have a there is a small risk of complete AV block in postoperative AV block pacemaker implantation may be considered for postoperative third-degree AV block that to normal AV node conduction in patients with forms of CHD which may AV block such as AV AV and Heart Heart Patients with CHD often have important and which influence both the indications for pacing as well as the of pacing Therefore, pacemaker implantation in these patients is not an isolated Bradycardia and are common following and in the absence of AV block, pacing is to ventricular pacemaker lead implantation may be considered at the of in patients with or evidence of conduction disease in heart with a progression to advanced heart regarding pacemaker implantation also the complexity of the patient's and Cardiac Cardiac sinus bradycardia is common and In rare cases, symptomatic sinus bradycardia may with at least for of sinus node of the that of heart patients years of required a pacemaker in the with need for pacing were and conduction disorders node or AV node may be to cardiac or Patients be for the of as late bradycardia may be the The of ICD implantation is not well established may be considered in patients who require and Cardiac Conduction and Cardiac Conduction include Kearns-Sayre and cardiac conduction are disorders with of the conduction either in or in with other such as neuromuscular and of conduction abnormalities may from AV block to complete AV block with an unpredictable by in the is a with cardiac conduction abnormalities often before the of heart the Kearns-Sayre with and has a risk for AV block and sudden cardiac an expert consensus statement on the evaluation and management of risk in neuromuscular disorders is Therefore, the recommendations may be to as data become In the of cases, is a limited disease and pacemaker implantation is not However, in some patients, recurrent may quality of and may in when the of is Therefore, in a select of patients who pacemaker may be useful by profound bradycardia or the of pacing on the clinical a between symptoms and bradycardia or be established to pacemaker Cardiac Cardiac text The of pacing as in the channelopathies is not well In patients with or of ventricular permanent pacemaker implantation may provide pacing has been to in infants with AV data also that pacing than the rate may the QT and the rate of recurrent in select long QT syndrome may cause or with bradycardia or complete AV In most cases, there is of AV However, in advanced heart block in is permanent and pacemaker implantation is data that who AV block due to syndrome will have of normal AV Introduction The following recommendations for ICD implantation are primarily based on adult guidelines, and with some to patients. ICD guidelines have been established based on a specific or risk for a sudden cardiac such as or cardiovascular In of pediatric sudden cardiac arrest (SCA) that in 50% of cases, the cause of the remains undefined an extensive Furthermore, in young patients with such as catecholaminergic polymorphic ventricular tachycardia or Brugada syndrome is often the initial of the Therefore, development of pediatric ICD recommendations based on specific cardiovascular be the following recommendations for ICD implantation will with considerations for young patients, followed by recommendations for ICD implantation when a specific cause or a defined risk for has been There remain extensive in current ICD recommendations, of age, for of the with in The recommendations that are based on limited clinical data or expert and consensus and require the of clinical and a shared decision for for The decisions regarding ICD implantation pediatric patients are made in the context of both the of device implantation as well as of the which may Therefore, a pediatric be in the ICD decision and the be by a or with in CIED implantation in the pediatric ICD implantation be a shared decision between the and This the and of an ICD on the patient's Furthermore, the indications for the ICD be at each with to current guidelines, a period of as of device may be considered in select ICD for Cardiac QT QT and are to risk when patients with may require ICD risk factors include the of symptoms at patients with or those with recurrent factors include a of with with and with are considered and cardiac in patients, in those with In addition, and cardiac ICD may be in selected ICD implantation in an with for a of is not clearly supported by published data and decision is in to of factors include previous of cardiac multiple and at ventricular on is also with Treatment with is with a in cardiac the of to patients may provide further In ICD implantation be for patients with or with ventricular on are in of patients with with cardiac recommended in patients who recurrent ICD In selected patients with as the initial of CPVT, cardiac ICD may be considered as a Brugada Brugada Although Brugada syndrome in the to may have with progression to The ICD remains the with for the management of ventricular or in patients with Brugada recommendations for risk including ventricular have been have not been in with risk of ventricular and in include in of the of symptoms or arrhythmogenic sinus node dysfunction and conduction abnormalities block or conduction implantation of an ICD is not in patients in the absence of risk factors. are to further risk factors and primary prevention ICD indications for pediatric patients with Brugada ICD for for rates in hypertrophic cardiomyopathy with rates between and have the for patients from SCD, of risk factors to primary prevention ICD implantation remains a the progression of the disease clinical practice guidelines risk for in by the of defined clinical risk However, pediatric that the of adult risk factors may in A pediatric that an was with SCA, a of pediatric a maximum or a with an increased risk of The of risk factors for SCD, such as late gadolinium on cardiac magnetic resonance and the of for specific remains and further before as specific risk factors for in pediatric patients with cardiomyopathy a of primary disorders with the of with This disorders such as arrhythmogenic ventricular and cardiac These are before and often overlap with other in of young patients with Patients with have a class ICD Although risk data are ICD implantation is reasonable in patients with VT, due to ventricular or an Heart and whether a external is reasonable be considered on an basis for those patients with advanced heart The of in pediatric patients with is which is than in adult Although have ICD survival benefit for prevention in pediatric NIDCM, the of has made to risk factors to recommendations for primary prevention ICD However, in to of adult patients with and there is no evidence that for primary prevention survival for pediatric patients with The of may overlap with other cardiomyopathies in of In the in the of at years was for to for left ventricular dysfunction a of were with in Therefore, factors which influence implantation of a primary prevention ICD include the the cardiomyopathy the of ventricular dysfunction and the of cardiac ICD for Heart ICD for Heart The between CHD and ventricular is well in of have risk factors for and including cardiac defects, abnormal and from of abnormalities or ablation of arrhythmogenic may ventricular function or these may be to or ICD placement may be in patients or at risk The of and and of ventricular dysfunction as risk factors for in CHD to be ICD implantation in patients with CHD intracardiac and This may require such as leads or Cardiac cardiac are devices which provide long term rhythm and provide of rhythm symptomatic monitoring an is recommended in symptomatic when are due to either or the to complete a For with provides the most for a and are considered the of when arrhythmogenic is not For may be useful in both of the bradycardia and correlation with clinical may also be useful for patients at risk for AV block in conditions such as Kearns-Sayre may be useful for arrhythmia in patients with cardiac arrhythmia and that need for changes in CIED CIED management the decisions of whether or not to CIED lead and of the and regarding lead management and were published in and in The following recommendations are to the guidelines with a on and patients with Although lead are rare for Therefore, lead be in with an to a comprehensive This and the to all A with CHD is to and There are extensive in regarding lead management in and patients with This limited data in the young and the of on and There is also absence of data regarding lead as on lead in the young do not based on adult lead management CIED CIED and follow-up both in-person evaluation and remote interrogation and monitoring of and The of monitoring are well established and include both of as well as of CIED and there are no consensus guidelines for CIED or in the pediatric Therefore, the following recommendations are based on on CIED with select recommendations regarding in with are also CIEDs and CIEDs and and in Patients with CIEDs provides comprehensive recommendations for individuals with both and and However, this document does not specific recommendations for patients with either or CIED For patients with CIED as there are no the is considered when with a in data that leads than small of in patients with both and leads that be in the of In the data on use in or leads are to provide specific recommendations or absolute the of data, also the of MRI, consideration of the of be made on a by CIEDs and CIEDs and of for patients with CIEDs to guidelines recommended for patients with or However, that patients with and in the ICD was and in The consisted of patients years including and there were no no and no injury In addition, the rate of lead was similar to rates in patients with CIEDs and the decision is including the cardiac disease and heart the and for device age, and of decision including the and other be utilized to the best of for individuals with CIEDs and The use of shared decision-making to all CIED implantation and clearly the and for the and their decisions will be for example, the decision to a permanent pacemaker to postoperative complete heart block in a who is pacemaker will be However, other such as implantation of an ICD for primary prevention of SCD, are and and include of ICD device and of risk of arrhythmia for the particular is is the use of for the primary prevention of in device and the development of novel lead for implantation in patients, the of patients at increased risk remains other important include are not limited to the of pacemaker implantation postoperative AV block, of patients with isolated who do not pacing, risk factors for and for lead implantation, and of with or is needed to criteria for of these new These include and conduction as well as data are to provide evidence for new and existing CIED be in with other relevant the and and for development of pediatric CIEDs and device to benefit young patients and their