Pacemaker Indications in Children

2021 PACES pediatric device consensus (21 years and under), with the 2023 HRS rows on pacing site and resynchronization

Irecommended
IIareasonable
IIbmay be considered
IIInot indicated (no benefit or harm)
LOE: B-NR nonrandomized data; C-LD limited data; C-EO expert opinion

Condition

Class

Isolated sinus node dysfunction (SND)

Class	Recommendation	LOE
I	Symptoms correlate with age-inappropriate bradycardia: atrial or dual-chamber pacemaker.	B-NR
I	Symptomatic SND caused by chronic medical therapy that has no alternative.	C-EO
IIa	Symptoms temporally associated with observed chronotropic incompetence: pacemaker with rate-responsive programming.	C-LD
IIb	Symptoms likely due to bradycardia or prolonged pauses, but a thorough investigation finds no conclusive correlation.	C-EO
III: No benefit	Asymptomatic SND.	C-EO
III: Harm	Symptomatic SND from a reversible cause.	C-EO

No rate cut-off; pacing mode

No cut-off: without symptoms of cerebral or systemic hypoperfusion, there is no minimum heart rate or maximum pause duration at which pacing is recommended. What matters most is a temporal correlation of symptoms with bradycardia for age.
Mode: in symptomatic SND, atrial-based pacing is generally recommended over single-chamber ventricular pacing. SND with CHD or a channelopathy: see those sections.

Isolated congenital complete atrioventricular block (CCAVB)

Class	Recommendation	LOE
I	Symptomatic bradycardia.	B-NR
I	Wide QRS escape rhythm, complex ventricular ectopy, or ventricular dysfunction.	B-NR
I	Asymptomatic neonate or infant with a mean ventricular rate ≤50 bpm. Rate alone should not be the criterion: low-output symptoms can occur at faster rates.	C-LD
IIa	Asymptomatic beyond the first year of life, with a mean ventricular rate <50 bpm or prolonged pauses.	B-NR
IIa	LV dilation (z score ≥3) with significant mitral insufficiency or systolic dysfunction.	C-LD
IIb	Asymptomatic adolescent with an acceptable ventricular rate, narrow QRS and normal ventricular function, after an individual risk/benefit review.	C-LD

Ages, rate measurement, natural history

Ages: newborn ≤30 days, infant ≤12 months.
Rate: judge heart-rate criteria on consistency, not a single measurement. Size (birth weight), CHD, ventricular function and comorbidities may weigh as much as the rate; poor feeding or tachypnea may mean low output.
Natural history: about 66% are paced in the first year of life and 90% by 20 years. Without pacing, LV dysfunction and mitral insufficiency progress, with cardiovascular deaths in the fourth or fifth decade.

Atrioventricular block: other considerations

Class	Recommendation	LOE
I	Clinically significant VT that is pause-dependent or associated with severe bradycardia; an ICD may be considered as a reasonable alternative.	C-LD
I	Symptomatic idiopathic advanced second- or third-degree AV block, not due to a reversible cause.	C-LD
IIa	Any degree of AV block that progresses to advanced second- or third-degree with exercise, without a reversible cause.	C-LD
IIb	Intermittent advanced second- or third-degree AV block, not due to a reversible cause, with minimal otherwise unexplained symptoms.	C-LD
III: Harm	Asymptomatic first-degree AV block or asymptomatic second-degree Mobitz type I.	C-LD

Exercise testing

Exercise test: helps locate the block. Supra-His block generally resolves with exercise; progression to advanced second- or third-degree block with exercise is rare, suggests His-Purkinje disease and carries a poor prognosis without a pacemaker. Apart from infiltrative or inflammatory causes, criteria are similar to CCAVB.

Postoperative atrioventricular block

Class	Recommendation	LOE
I	Advanced second- or third-degree AV block persisting at least 7–10 days after cardiac surgery.	B-NR
I	Late-onset advanced second- or third-degree AV block, especially after transient postoperative AV block.	C-LD
IIb	Unexplained syncope after transient postoperative advanced second- or third-degree AV block.	C-LD
IIb	At <7 postoperative days when the block is not expected to resolve because of extensive conduction-system injury.	C-EO
IIb	Selected patients with transient postoperative advanced second- or third-degree AV block who are predisposed to progressive conduction abnormalities (below).	C-EO

Recovery by day; who is predisposed

Frequency: AV block complicates 3%–8% of congenital heart operations; 1%–3% of patients need a permanent pacemaker.
Recovery: of those who regain conduction, ≥85% do so by postoperative day 7 and ≥95% by day 10. Late complete block can still follow transient block, months to decades later.
Predisposed (supporting text): new postoperative bifascicular block after transient block (limited data); discordant AV connections, AV septal defects, heterotaxy syndromes.

Congenital heart disease: specific considerations

All the rows for structurally normal hearts apply; in addition:

Class	Recommendation	LOE
I	CCAVB in a neonate or infant with complex CHD, with bradycardia associated with hemodynamic compromise or a mean ventricular rate <60–70 bpm.	C-LD
IIa	Recurrent intra-atrial re-entrant tachycardia when ablation or medication is ineffective or not acceptable: pacemaker with atrial antitachycardia pacing.	B-NR
IIa	Impaired hemodynamics from sinus bradycardia or loss of AV synchrony: atrial or dual-chamber pacemaker.	C-LD
IIa	Tachy-brady syndrome with symptoms from pauses due to sudden-onset bradycardia: atrial or dual-chamber pacing.	C-LD
IIa	Complex CHD with sinus or junctional bradycardia: mean awake resting heart rate <40 bpm or prolonged pauses in the ventricular rate.	C-EO
IIb	Simple or moderate CHD with sinus or junctional bradycardia: mean awake resting heart rate <40 bpm or prolonged pauses.	C-EO
III: Harm	Avoid endocardial leads in CHD with an intracardiac shunt, except in selected cases after an individual risk/benefit review. If used, anticoagulation is mandatory and thromboembolism remains a risk.	B-NR

What counts as complex; leads and mode

Complex: palliative repairs, or impaired ventricular function or circulatory physiology (PACES cites the 2018 AHA/ACC adult CHD guideline for complexity).
Leads: lost vascular access or no direct access to the cardiac chambers, or persistent right-to-left shunting, needs epicardial leads. Without high-grade AV block, atrial pacing is preferred to avoid pacing-induced ventricular dysfunction.
At surgery: a prophylactic pacemaker or lead may be considered with conduction disease in defects known to progress to advanced block (discordant AV connections, heterotaxy).

After heart transplant

Class	Recommendation	LOE
I	Persistent symptomatic bradycardia not expected to resolve, and any other Class I pacing indication.	C-LD
IIa	Marked chronotropic incompetence impairing quality of life, late after transplant.	C-LD
IIb	Relative bradycardia that is prolonged or recurrent, or limits rehabilitation or discharge, after postoperative recovery.	C-LD
IIb	Any degree of AV block thought to be due to graft vasculopathy.	C-LD

Early and late bradycardia

Early: transient sinus bradycardia is common and usually resolves; allow at least a week for sinus node recovery. About 1% of recipients under 18 years needed a pacemaker early (UNOS, 1994–2014).
Late: bradycardia may be the first sign of transplant coronary artery disease; evaluate for it.

Neuromuscular diseases and other progressive conduction diseases

Class	Recommendation	LOE
I	Neuromuscular disease with symptomatic bradycardia from SND or any degree of AV block.	B-NR
I	Kearns-Sayre syndrome: any degree of AV block (including first-degree) and/or conduction abnormality, because progression is unpredictable.	C-LD
IIa	Myotonic dystrophy type 1: PR >240 ms or native QRS >120 ms. Additional defibrillator capability may be considered.	B-NR
IIa	Lamin A/C gene mutations (including limb-girdle and Emery-Dreifuss muscular dystrophies): PR >240 ms and/or left bundle branch block. Additional defibrillator capability may be considered.	C-LD
IIb	Any progressive conduction disease that could rapidly worsen AV nodal function, even with normal AV conduction, weighing age, size and other individual risk factors.	C-LD

Conditions covered; newer HRS statement

Conditions: Duchenne, Becker, myotonic dystrophy type 1, Friedreich ataxia, Emery-Dreifuss, facioscapulohumeral muscular dystrophy, Barth syndrome, Kearns-Sayre syndrome, lamin A/C mutations, desmin-related myopathies.
PR >240 ms: reported as a predictor of progressive AV block and/or ventricular arrhythmias in Adult patients with myotonic dystrophy and laminopathy.
Newer: the 2022 HRS neuromuscular consensus, which PACES anticipated, has its own graded recommendations for many of these disorders; they are not reproduced here.

Neurocardiogenic syncope

Class	Recommendation	LOE
IIa	Severe recurrent breath-holding spells with a documented cardioinhibitory response on ECG monitoring, complicated by prolonged syncope, prolonged postanoxic convulsions or other bradycardia-induced symptoms.	B-NR
IIb	Recurrent symptomatic neurocardiogenic syncope with documented spontaneous bradycardia or asystole, after other medical treatments have failed.	C-LD
IIb	Epilepsy with severe symptomatic (ictal-induced) bradycardia not improved by antiepileptic therapy.	C-LD
III: No benefit	A positive cardioinhibitory tilt response alone.	C-EO
III: Harm	Neurocardiogenic syncope in which hypotension is the major or a significant component.	C-EO

Documenting the link

Document: bradycardia or asystole during clinical syncope, ideally more than once (event monitor or insertable cardiac monitor).
Pallid breath-holding: complete resolution or a significant reduction of events in 86% with pacing (mostly infants and toddlers).

Cardiac channelopathies

Class	Recommendation	LOE
I	Pause-dependent, clinically significant VT; an ICD may be considered as a reasonable alternative.	C-LD
IIb	Long QT syndrome with functional 2:1 AV block: pacemaker as adjunctive therapy.	C-LD
IIb	Long QT syndrome or another channelopathy in which a faster heart rate may reduce the arrhythmia burden or bradycardia symptoms: adjunctive pacing.	C-LD
III: No benefit	Atrial pacing alone in complete atrial standstill (high chance of noncapture).	C-LD

SND or AV block with a channelopathy

SND or AV block from the channelopathy or from its drugs: use the sinus node and AV block rows. ICD rows by channelopathy: ICD Indications.

Inflammation and infection

Class	Recommendation	LOE
I	High-grade or symptomatic AV block from a known, potentially reversible cause that persists despite treatment of the cause.	C-LD
IIa	Chagas disease with advanced second- or third-degree AV block (spontaneous resolution unlikely); an ICD may be a reasonable alternative.	C-LD
III: No benefit	Acute AV block from a known reversible cause, with recovery of normal AV conduction.	C-EO

Reversible causes: how long to wait

Lyme: of those with symptomatic Lyme AV block, about 40% may need temporary pacing; block usually reverses with antibiotics. Acute myocarditis: 67% of young patients recover conduction within 7 days; monitor late for recurrence.
Potentially reversible causes: maximize treatment of the cause (antibiotics, steroids, intravenous immunoglobulins); if tolerated, waiting up to several months before implant is warranted. Advanced AV block in Chagas disease is permanent, and pacing is indicated.
MIS-C: transient AV conduction abnormalities reported in MIS-C with ventricular dysfunction.

Pacing site and resynchronization (2023 HRS/APHRS/LAHRS)

Pacing site, CRT and conduction system pacing, not bradycardia indications. Classes as printed (2a = IIa, 2b = IIb).

Class	Recommendation	LOE
2a	CHD, systemic LV, on GDMT, LVEF <45% and dyssynchrony (QRS duration z score ≥3 or ventricular pacing ≥40%): CRT with BiV pacing, to reduce death or transplant.	C-LD
2a	CHD, systemic single ventricle, needing pacing: apical rather than nonapical pacing.	C-LD
2b	CHD, systemic single ventricle, with symptomatic HF on GDMT: CRT with multisite ventricular pacing.	C-LD
2b	CHD, systemic RV, with symptomatic HF on GDMT associated with ventricular electrical delay or requiring substantial ventricular pacing: CRT with BiV pacing.	C-LD
2b	CHD, subpulmonary RV, with RV dysfunction and RBBB: CRT with fusion-based pacing, to improve RV function.	C-LD
2b	CCTGA with AV block, no anatomic repair: CSP with HBP or LBBAP.	C-LD
2a	Pediatric (no CHD), complete AV block, preexisting ventricular pacing, symptomatic clinical HF on GDMT: CRT with BiV pacing.	C-LD
2b	Pediatric (no CHD), complete AV block, clinical HF on GDMT: CPP.	C-LD
2a	Pediatric (no CHD), pacemaker for AV block: RV mid-septal, inflow or outflow tract transvenous endocardial site, or apical LV (systemic ventricle) epicardial pacing, rather than RV apical endocardial or epicardial sites.	C-LD

Abbreviations; conduction system pacing in children

Abbreviations: CRT cardiac resynchronization therapy; BiV biventricular; CPP cardiac physiologic pacing (CRT with BiV pacing or CSP); CSP conduction system pacing; HBP His bundle pacing; LBBAP left bundle branch area pacing; GDMT guideline-directed medical therapy; HF heart failure; CCTGA congenitally corrected transposition of the great arteries.
Children: the guideline judges data too limited for recommendations on His bundle or left bundle branch area pacing in pediatric patients who need a pacemaker (Section 7.2.2). CCTGA carries a 2% annual risk of AV block.

Key points

Relative, not absolute, rate: correlate symptoms with bradycardia for age (45 bpm can be normal in an adolescent, profound bradycardia in an infant).
CCAVB: Class I at ≤50 bpm in an asymptomatic neonate or infant, or <60–70 bpm in a neonate or infant with complex CHD; rate alone should not decide.
Postoperative block: wait at least 7–10 days, unless extensive conduction-system injury makes recovery unlikely (IIb).
Intracardiac shunt: avoid endocardial leads (III: Harm).
After implant (Class I): in-person check and remote monitoring within 2–4 weeks, remote monitoring every 3–12 months, in-person at least yearly. Echo every 1–3 years if >40% ventricular paced (IIa).
Share the decision with the patient and family (Class I).

Sources and how this page was checked

Sources

Shah MJ, Silka MJ, Silva JNA, et al. 2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients. Heart Rhythm 2021;18(11):1888-1924. doi:10.1016/j.hrthm.2021.07.038. Co-published in JACC Clin Electrophysiol 2021;7(11):1437-1472, Cardiol Young 2021;31(11):1738-1769 and Indian Pacing Electrophysiol J 2021;21(6):367-393 (open access); executive summary also in Ann Pediatr Cardiol 2022;15(3):323-346 (open access). Pediatric means 21 years and under. Section 2 (permanent pacemakers), all rows; key points also use Sections 6.1 and 7.4. It supersedes the pediatric device recommendations of the 2008 ACC/AHA/HRS guideline and its 2012 update.
Chung MK, Patton KK, Lau CP, et al. 2023 HRS/APHRS/LAHRS guideline on cardiac physiologic pacing for the avoidance and mitigation of heart failure. Heart Rhythm 2023;20(9):e17-e91. doi:10.1016/j.hrthm.2023.03.1538. Section 7 (congenital heart disease and pediatric populations), all rows. PACES 2021 left resynchronization and physiologic pacing to this document.
Groh WJ, Bhakta D, Tomaselli GF, et al. 2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Heart Rhythm 2022;19(10):e61-e120. doi:10.1016/j.hrthm.2022.04.022. Named only; its recommendations are not on this page.

How it was checked

PACES 2021 was read in full (Indian Pacing Electrophysiol J copy, Europe PMC PMC8577100), and every Section 2 row, class and level of evidence was compared with the executive summary (Ann Pediatr Cardiol, PMC9802608); they match. The Heart Rhythm typeset copy could not be opened.
Section 2 holds 50 recommendations: 15 Class I, 13 IIa, 14 IIb, 4 III: Harm, 4 III: No benefit. All 50 are on this page, grouped and ordered as in the document (Sections 2.2 to 2.11).
The 2023 guideline was read in full (Heart Rhythm copy, PMC11062890) and its 9 Section 7 rows (4 Class 2a, 5 Class 2b, all C-LD) were compared with the Journal of Arrhythmia co-publication (PMC10549836); they match.
Wording is condensed, not quoted; class, level of evidence and qualifiers are kept. Names and abbreviations follow each document. Check the source for exact wording.
As printed: the infant CCAVB row reads ≤50 bpm and the row beyond the first year <50 bpm. The pause-dependent VT row appears twice in PACES (AV block, Section 2.4, and channelopathies, Section 2.10), with the same class and level of evidence; both are shown. PACES capitalizes “No Benefit” only in the sinus node table. Its Section 2.1 says indications in children and adolescents (under 19 years) are often similar to adults, while the document defines pediatric as 21 years and under.
Not reproduced: PACES Sections 4 (insertable cardiac monitors), 5 (lead management), 6 (follow-up, apart from the key points) and 7 (MRI, sports, low- and middle-income countries); the ICD page carries the sports and shared decision-making rows. A 2026 multisociety update on lead management and extraction updates the 2017 statement that Section 5 drew on.
As of October 2026 no newer pediatric pacemaker-indication document was found. The 2018 ACC/AHA/HRS bradycardia guideline (over 18 years) and the 2025 ACC/AHA adult congenital heart disease guideline are adult documents.