ICD Indications in Children

2021 PACES pediatric device consensus (21 years and under), with the 2024 AHA/ACC recommendations for HCM

Before any ICD decision

Survival: every recommendation assumes meaningful survival of more than 1 year, with reasonable quality of life and function.
HCM: use the 2024 AHA/ACC rows below; they are newer than PACES 2021.
Who decides: involve a pediatric cardiologist and an implanter with pediatric device experience; share the decision with the patient and family.
Unexplained arrest is common: no cause is found in about half of pediatric arrest survivors despite a full evaluation.
Revisit the indication at each reintervention, especially after nonuse; stopping device therapy may be considered in selected cases.

Shared decision, device type

Shared decision covers: age and size, need for an epicardial system, beliefs, quality of life, psychological impact.
Device type: PACES 2021 makes no recommendation specific to the subcutaneous ICD or the wearable defibrillator; both appear only in its supporting text. The 2024 HCM guideline has device-selection recommendations (including the subcutaneous ICD), not reproduced here.

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

General (secondary prevention and limits)

Class	Recommendation	LOE
I	Survivor of sudden cardiac arrest from VT/VF, once completely reversible causes are excluded and an ICD is judged more beneficial than other treatments that could substantially lower the risk.	B-NR
IIb	Sustained VT that medication and/or catheter ablation cannot adequately control.	C-EO
IIb	Primary prevention in genetic cardiovascular disease with risk factors for arrest, or with a pathogenic variant and a family history of recurrent arrest.	C-EO
III: Harm	Incessant ventricular tachyarrhythmia (risk of ICD storm).	C-EO
III: Harm	Ventricular arrhythmias that medication and/or ablation already treat adequately.	C-LD
III: Harm	Expected survival under 1 year, even when other criteria are met.	C-EO
III: Harm	Endocardial leads with an intracardiac shunt, except in selected cases after an individual risk-benefit review. If used, anticoagulation is mandatory and thromboembolic risk remains.	B-NR

Long QT syndrome

Class	Recommendation	LOE
I	Survivor of sudden cardiac arrest: ICD plus a beta-blocker. In selected patients, medical therapy and/or cardiac sympathetic denervation may be an alternative.	B-NR
I	Symptomatic (arrhythmic syncope or VT) when beta-blockers are ineffective or not tolerated, and sympathetic denervation or other drugs are not effective alternatives.	B-NR
IIb	Primary prevention with established clinical risk factors and/or pathogenic variants (see below).	C-LD
III: Harm	Asymptomatic and judged low risk, without a trial of beta-blocker.	C-LD

Higher-risk features (supporting text): symptoms before age 10, prior arrest, recurrent syncope; QTc ≥550 ms with any genotype; QTc ≥500 ms with LQT1; LQT2 in females; LQT3 in males.
May be at highest risk: Jervell and Lange-Nielsen, Timothy syndrome, calmodulinopathies. Also at significant risk: infants presenting with bradycardia, functional 2:1 AV block or cardiac arrest.

Family history

Family history alone: an ICD for an asymptomatic low-risk patient because of LQTS-related sudden death in the family is not clearly supported; decide individually.

Catecholaminergic polymorphic VT (CPVT)

Class	Recommendation	LOE
I	Cardiac arrest or arrhythmic syncope despite a maximally tolerated beta-blocker plus flecainide and/or sympathetic denervation.	C-LD
IIa	Aborted arrest as the first presentation: ICD together with drug therapy, with or without denervation. Drugs and/or denervation without an ICD may be considered instead.	C-LD
IIb	Polymorphic or bidirectional VT despite optimal drug therapy, with or without denervation.	C-LD
III: Harm	Asymptomatic CPVT.	C-EO

ICD programming

Programming: inappropriate shocks occur in 20–30% of CPVT patients with ICDs; program to treat VF while limiting inappropriate shocks and the risk of electrical storm.

Brugada syndrome

Class	Recommendation	LOE
I	Survivor of sudden cardiac arrest, or documented spontaneous sustained VT.	B-NR
IIa	Spontaneous type 1 Brugada ECG pattern with recent syncope presumed arrhythmic.	B-NR
IIb	Syncope presumed arrhythmic, with a type 1 pattern seen only after provocative drugs.	C-EO
III: No benefit	Asymptomatic without risk factors.	C-EO

Higher-risk features in children

Higher risk in children, most important first: symptoms (arrest or arrhythmic syncope), spontaneous type 1 ECG, atrial arrhythmias and/or sinus node dysfunction, conduction disease (AV block or intraventricular conduction delay).

Hypertrophic cardiomyopathy (2024 AHA/ACC)

HCM Risk-Kids (1 to 16 years) and HCM Risk-SCD are on the SCD Risk calculator, which applies the pediatric rows under 16 years and the adult rows from 16.

Class	Recommendation	LOE
I	Previous documented cardiac arrest or sustained VT: ICD recommended (any age).	B-NR
IIa	Children: one or more conventional risk factors (unexplained syncope, massive LVH, NSVT, family history of early HCM-related sudden death): ICD reasonable after weighing the relatively high long-term complication rates in younger patients.	B-NR
IIa	Adults: one or more major sudden death risk factors: reasonable to offer an ICD.	B-NR
IIa	With one or more major risk factors, discussing the estimated 5-year sudden death risk and mortality can help shared decision-making.	B-NR
IIb	Children: additional factors such as extensive LGE on contrast CMR and systolic dysfunction can be useful in ICD shared decision-making.	B-NR
IIb	Selected adults: no major risk factor, or still uncertain after assessment: an ICD may be considered with extensive LGE on CMR or NSVT on ambulatory monitoring.	B-NR
I	Use individual clinical judgment and a thorough, balanced discussion of the evidence, benefits and estimated risks so the patient takes part in the ICD decision.	C-EO
III: Harm	HCM without risk factors: no ICD.	B-NR
III: Harm	An ICD only to allow competitive sports: do not place.	B-NR

LVH in children (z scores)

PACES text: express wall thickness as a z score. A posterior wall z score ≥5 was associated with VT/VF or arrest in a multicenter study; a meta-analysis linked maximal wall thickness ≥30 mm or z score ≥6 with higher sudden death risk.

PACES 2021 HCM rows (superseded by 2024)

Class	Recommendation	LOE
I	Survivor of arrest or spontaneous sustained VT.	B-NR
IIa	Children with one or more primary risk factors (unexplained syncope, massive LVH, NSVT, family history of early HCM-related sudden death), after considering long-term ICD complications.	B-NR
IIb	No primary risk factor but secondary ones such as extensive LGE on CMR or systolic dysfunction.	B-NR*
III: Harm	HCM genotype alone, without known pediatric risk factors.	C-LD

*B-NR in the full document; the executive summary prints C-LD.

Arrhythmogenic cardiomyopathy (including ARVC)

Class	Recommendation	LOE
I	Resuscitated from sudden cardiac arrest, or sustained VT that is not hemodynamically tolerated.	B-NR
IIa	Hemodynamically tolerated sustained VT, syncope presumed arrhythmic, or LVEF ≤35%.	B-NR
IIb	Inherited arrhythmogenic cardiomyopathy with increased sudden death risk on assessment of additional risk factors.	C-LD

Scope; advanced heart failure

Scope: includes arrhythmogenic right or left ventricular cardiomyopathy, lamin A/C, filamin C, phospholamban and cardiac amyloid; myocarditis, Chagas disease and sarcoidosis may also fit.
Advanced heart failure: weigh transplant candidacy and timing, and whether a wearable defibrillator is a reasonable alternative.

Nonischemic dilated cardiomyopathy

Class	Recommendation	LOE
I	Survived sudden cardiac arrest, or sustained VT not from a completely reversible cause.	B-NR
IIb	Syncope, or LVEF ≤35%, despite optimal medical therapy (see Primary prevention below).	C-LD
III: Harm	Medication-refractory advanced heart failure in a patient who is not a transplant or VAD candidate.	C-EO
III: No benefit	Advanced heart failure, urgently listed for transplant and staying in hospital until transplant, even if other criteria are met.	C-EO

Primary prevention, LV noncompaction, restrictive CM

Primary prevention: unlike some adult studies, there is no clear evidence that a primary-prevention ICD for LVEF ≤35% improves survival in children; weigh complications, transplant timing and a wearable defibrillator.
LV noncompaction: no separate recommendation. Sudden death at 15 years was 23% with LV noncompaction vs 5% with idiopathic dilated cardiomyopathy; etiology, phenotype, degree of dysfunction and arrhythmias guide the decision.
Restrictive cardiomyopathy: no recommendation of its own (PACES covers it under its HCM and general rows). The text says an ICD may be appropriate with heart failure or unexplained syncope when transplant is not immediate.

Congenital heart disease

Class	Recommendation	LOE
I	Survivor of sudden cardiac arrest, after evaluation for the cause and exclusion of completely reversible causes.	B-NR
I	Hemodynamically unstable sustained VT, after hemodynamic and electrophysiologic evaluation. Ablation or surgical repair may be alternatives in carefully selected patients.	C-LD
IIa	Systemic LVEF <35% with sustained VT or presumed arrhythmic syncope.	C-LD
IIb	Spontaneous hemodynamically stable sustained VT, after hemodynamic and electrophysiologic evaluation. Ablation or surgical repair may be alternatives in carefully selected patients.	C-EO
IIb	Unexplained syncope with ventricular dysfunction, NSVT, or ventricular arrhythmia inducible at EP study.	C-LD
IIb	Single or systemic right ventricle with EF ≤35%, especially with VT, arrhythmic syncope or severe systemic AV valve regurgitation.	C-EO

Tetralogy; expected shocks and complications

Tetralogy and others: no lesion-specific recommendation. Residual lesions, abnormal hemodynamics and surgical scar raise the risk; repairing residual lesions or ablating the substrate may help but often does not remove the risk.
Expect: appropriate shocks 3–6% a year, more often with secondary-prevention indications; complications as high as 26–45%; frequent inappropriate shocks. Anatomy, shunts or limited venous access may need epicardial leads, nontransvenous coils or a subcutaneous ICD.

Other ICD-related recommendations, monitoring and sports (PACES 2021)

Class	Recommendation	LOE
I	Channelopathy with pause-dependent, clinically significant VT: pacemaker indicated; an ICD may be a reasonable alternative.	C-LD
IIa	Myotonic dystrophy type 1: pacemaker reasonable for PR >240 ms or native QRS >120 ms; defibrillator capability may be considered.	B-NR
IIa	Lamin A/C variants (including limb-girdle and Emery-Dreifuss): pacemaker reasonable for PR >240 ms and/or LBBB; defibrillator capability may be considered.	C-LD
IIa	Chagas disease with advanced second- or third-degree AV block: pacemaker reasonable; an ICD may be a reasonable alternative.	C-LD
I	Insertable cardiac monitor: syncope with high-risk features when a comprehensive evaluation finds no cause or no specific treatment, and there is no conventional pacemaker or ICD indication.	B-NR
I	Remote monitoring: every 3–6 months for ICDs (3–12 for pacemakers); every 1–3 months as a device nears elective replacement.	C-EO
I	Sports: base participation decisions on the diagnosis and physiology, not on the presence of the device.	C-EO
IIa	Sports: with a pacemaker or ICD, competitive sports or intense recreational exercise is reasonable after shared decision-making with a provider who explains the risk, including coaches, schools and teams.	C-LD
III: No benefit	Sports: do not place an ICD solely so that a patient can compete.	B-NR
I	Shared decision-making among patient, family, provider and other stakeholders before care plans, covering risks, benefits, alternatives and expected outcomes before and after implant.	B-NR

After transplant; conditions without rows

After heart transplant: no recommendation. The role of a prophylactic ICD is not well established, but it may be considered when a pacemaker is needed, weighing coronary vasculopathy and LV dysfunction.
No condition-specific rows: short QT, idiopathic VF and myocarditis. The General rows (for example after an arrest) still apply, and myocarditis may fall under arrhythmogenic cardiomyopathy.

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). Pediatric means 21 years and under. Section 3 (ICDs), plus the ICD-related rows of Sections 2.8.1, 2.10, 2.11, 4, 6.1, 7.2 and 7.4. It supersedes the pediatric device recommendations of the 2008 ACC/AHA/HRS guideline and its 2012 update.
Ommen SR, Ho CY, Asif IM, et al. 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR guideline for the management of hypertrophic cardiomyopathy. Circulation 2024;149(23):e1239-e1311. doi:10.1161/CIR.0000000000001250. Recommendations for ICD placement (the device-selection recommendations are not reproduced).

How it was checked

PACES 2021 was read in full (Indian Pacing Electrophysiol J copy, Europe PMC PMC8577100) and every ICD row, class and level of evidence was compared with the Cardiology in the Young copy; they match. The Heart Rhythm typeset copy could not be opened.
Section 3 holds 36 ICD recommendations: 10 Class I, 5 IIa, 11 IIb, 8 III: Harm, 2 III: No benefit. All 36 are on this page (the 4 HCM rows under "superseded").
Wording is condensed, not quoted. Class, level of evidence and the qualifiers are kept, and an independent review compared every row with the source text; check the source for exact wording.
Known differences between copies: the PACES HCM IIb row is B-NR in both full documents but C-LD in the executive summaries. The CHD IIa row prints LVEF <35% where the other rows use ≤35%; it is shown as printed.
As of October 2026 no newer pediatric device consensus was found. Adult documents (2025 ACC/AHA adult congenital heart disease guideline, 2025 appropriate use criteria for ICDs) do not replace it for children.