Repaired Tetralogy of Fallot: Imaging Follow-up and RVOT Intervention

Imaging intervals, deciding thresholds and pulmonary valve replacement, child to adult

Age

How often to test

Surveillance testing (AHA 2024 Table 3)
Test	≤12 y	13–18 y	Adult, stage A / B / C / D (mo)
Echo	Every 12–24 mo	Every 12–24 mo	24–36 / 24 / 12 / 6–12
CMR	Baseline at 12 y	Every 4–5 y	36 / 24–36 / 12–24 / 12
Exercise test (CPET)	Baseline at 12 y	Every 4–5 y	36–60 / 24–60 / 12–24 / 12–24
ECG	Every 12 mo	Every 12 mo	24 / 12 / 12 / 6–12
Holter	By symptoms	By symptoms	By symptoms (A, B); 12–24 (C, D)
Cardiac CT	As needed

Other schedules
Age	Schedule	Source
Any age	Echo every 12 mo to 9 y, then 24 mo. CMR from 10 y: every 36 mo if stable, 12 mo with moderate (≥150 mL/m²) or progressive (>25 mL/m² rise) RV dilatation, or RV EF ≤48% or a ≥6% fall	ASE 2014 Table 6
Adult	CMR every 3–5 y (normal ventricular function, PR ≤mild); every 2–3 y (ventricular dysfunction and/or PR ≥moderate)	ACHD 2025

What each test should report; exercise-test timing

Echo (ASE 2014, AHA 2024): RVOT and branch PAs (narrowest segment, gradients, aneurysm), PR grade, TR grade and mechanism, RV pressure from TR jet, RV size and function, residual VSD, LV size and function, aortic root and ascending aorta. Primary tool in childhood; complementary from adolescence as windows narrow.
CMR: reference standard for ventricular volumes and function and for flows (PR fraction, branch PA flow split, Qp:Qs); also ventricular mass, aorta, proximal coronary arteries and LGE (AHA 2024, ASE 2014, ESC 2020). Gadolinium at the first study, if >3 years since the last LGE, or with clinical or functional deterioration (ASE 2014).
CT: when CMR is contraindicated or degraded by metal, for coronary anatomy, and before transcatheter valves (AHA 2024, ESC 2020).
Exercise test (STS 2026 exercise paper, expert opinion): no evidence-based interval; yearly if near the PVR threshold (low activity, RV dilation or dysfunction), every 3 years with excellent function and minimal RV dilation; a meaningful change is at least 10% to 20%; repeat with the same modality (treadmill or cycle).

Measurements that decide

Thresholds as printed
Group	Threshold	Source
PR fraction (CMR)
Any age	Predominant PR ≥25% with RVOT gradient ≤25 mm Hg or RVSP ≤1/3 systemic	AHA 2024 Fig 2
Adult	Moderate ≥25%	ACHD 2025; STS 2026
Adult	Severe >30–40%	ESC 2020
RV end-systolic volume index (RVESVi)
Any age	PVR benefit greatest >80 mL/m² (one multicenter study)	AHA 2024
Adult	>80 mL/m²	ACHD 2025
Adult	≥80 mL/m²	STS 2026; ESC 2020
RV end-diastolic volume index (RVEDVi)
Any age	Earlier guidelines: usually 150–160 mL/m²; clinical benefit unclear	AHA 2024
Adult	≥160 mL/m²	STS 2026; ESC 2020
Adult	RV EDV ≥2× LV EDV	ACHD 2025
RV ejection fraction (RVEF)
Any age	≤46%	AHA 2024
Adult	≤46%	ACHD 2025
Adult	<47%	STS 2026
LV ejection fraction (LVEF)
Any age	≤50%	AHA 2024
Adult	≤50%	ACHD 2025
Adult	<55%	STS 2026
RV pressure and RVOT obstruction
Any age	RVSP ≥2/3 systemic (risk criterion); gradient ≥60 mm Hg = predominant stenosis	AHA 2024
Any age	Echo peak velocity <3 / 3–4 / >4 m/s: mild / moderate / severe (normal cardiac output)	ASE 2014
Adult	RVSP >2/3 systemic	ACHD 2025
Adult	At least moderate: peak velocity >3 m/s; no symptoms: RVSP >80 mmHg	ESC 2020
Exercise capacity
Any age	Peak VO₂ ≤70% predicted for age and sex	AHA 2024
Adult	Declining exercise capacity	ACHD 2025; ESC 2020
Tricuspid regurgitation (TR)
Any age	TR fraction ≥20%: significant residual lesion	AHA 2024
Adult	Progressive functional TR ≥moderate with RV dilation	ACHD 2025
Adult	Progression to ≥moderate	ESC 2020
Branch PA stenosis
Any age	Stenosis or hypoplasia with ≤30% flow	AHA 2024
Child	Gradient >20–30 mm Hg, RV or proximal main PA pressure >½–⅔ systemic, or flow split 35%/65% or worse	AHA 2011
Adult	Isolated stenosis: <35% flow, gradient >20–30 mm Hg, RV or proximal mean PA pressure >½ systemic	ACHD 2025
QRS duration
Any age	≥180 ms: 1 point in two risk scores (575 adults; cases’ mean age 27 y) abstract	AHA 2024 Table 2
Adult	≥180 ms: VT and sudden-death risk factor	ACHD 2025 Table 32; ESC 2020
RV longitudinal strain (echo)
Any age	Change >3 percentage points: get CMR	AHA 2024

Echo signs of severe PR; echo RV cut-offs (adult)

Severe PR: diastolic flow reversal in the main or branch PAs with a jet ≥50% of the annulus; jet/annulus >0.7 identified CMR PR fraction ≥40%; pressure half-time <100 ms (unreliable with high RV end-diastolic pressure). PR index <0.77 identified PR fraction >25% (“significant”). Free PR is laminar and easy to miss on colour (ASE 2014). Grading tables: Valve Regurgitation.
Adult RV dilated at base >42 mm, mid >35 mm (not indexed); TAPSE lower limit 16 mm; S′ <10 cm/s; RV wall >5 mm = hypertrophy; RA area >18 cm². TR vena contracta >0.7 cm = severe. Pediatric normals not available (ASE 2014).
RV end-diastolic area <20 cm²/m² was associated with CMR RVEDVi <170 mL/m² (one study; population not stated). FAC lower reference value 35% (population not stated). 2D echo correlates only modestly with CMR, and 3D underestimates volumes (ASE 2014).

When to replace the pulmonary valve

AHA 2024 framework (no class): consider PVR when

RVOT dysfunction plus symptoms and ≥1 risk criterion, or no symptoms and ≥2 .
Risk criteria: INDICATOR score ≥3 ; RVEF ≤46%; LVEF ≤50%; peak VO₂ ≤70% predicted; RVSP ≥2/3 systemic.

Class	Recommendation	Level, source
I	Symptoms attributable to moderate or greater pulmonary insufficiency (children and adults): PVR recommended	B-NR STS 2026
IIa	Child RV-to-PA conduit with moderate to severe PR or stenosis: percutaneous PVR if device criteria are met	B AHA 2011

STS 2026 pediatric expert consensus (13 statements; abstract): not reproduced here.

Adult guidelines

Class	Recommendation	Level, source
1	Symptoms, moderate or greater PV dysfunction : PVR, surgical or transcatheter	B-NR ACHD 2025
2a	No symptoms, moderate or greater PV dysfunction, ≥2 of: RVESVi >80 mL/m², RV EDV ≥2× LV EDV, RVEF ≤46%, LVEF ≤50%, declining exercise capacity	B-NR ACHD 2025
2a	Moderate or greater PV dysfunction with progressive ventricular systolic dysfunction	C-LD ACHD 2025
2b	Moderate or greater PV dysfunction, progressive functional TR ≥moderate, RV dilation: pulmonary valve intervention	C-LD ACHD 2025
2b	Moderate or greater PR with ventricular tachyarrhythmia needing treatment: PVR with the arrhythmia intervention	C-LD ACHD 2025
IIa	No symptoms, regurgitant fraction ≥25%, ≥2 of: RVEDV ≥160, RVESV ≥80 mL/m², RVEF <47%, LVEF <55%	B-NR STS 2026
IIb	Same, with RVESV ≥80 mL/m² alone	B-NR STS 2026
I	Symptoms, severe PR and/or at least moderate RVOT obstruction (peak velocity >3 m/s): PVR	C ESC 2020
IIa	No symptoms, severe PR and/or RVOT obstruction, any 1 of: lower objective exercise capacity; RV dilating to RVESVi ≥80 and/or RVEDVi ≥160 mL/m², and/or TR progressing to ≥moderate; progressive RV dysfunction; RVSP >80 mmHg	C ESC 2020
I	No native outflow tract : catheter intervention (TPVI) preferred if anatomically feasible	C ESC 2020

Adult imaging, catheter and arrhythmia recommendations (ACHD 2025, ESC 2020, STS 2026)

Class	Recommendation	Level, source
1	Echo for PV and TV dysfunction, RA size, ventricular size and function, residual VSDs and RV systolic pressure	B-NR ACHD 2025
1	With residual sequelae, CMR for PV function, ventricular size and function, branch PA size and flow, RVOT and LVOT anatomy, residual shunts or collateral flow, and fibrosis	B-NR ACHD 2025
1	Before conduit stenting or a balloon-expandable transcatheter valve: assess coronary compression risk	B-NR ACHD 2025
1	Native RVOT considered for transcatheter PVR: cardiac CT for anatomic suitability	B-NR ACHD 2025
2a	Moderate risk of sustained VT or sudden death: invasive electrophysiology study	B-NR ACHD 2025
2a	Noninvasive data inadequate (arrhythmia, heart failure, unexplained ventricular dysfunction, suspected PH, residual lesions, cyanosis): catheterization with angiography	C-EO ACHD 2025
2b	Before PV placement: invasive electrophysiology study with programmed stimulation and 3D mapping for latent monomorphic VT substrate	C-LD ACHD 2025
2b	12-lead ECG, contrast-enhanced MR and/or CT angiography to characterize VT substrate	C-LD ACHD 2025
2a	High sudden-death risk (clinical markers and/or score, Tables 32–33): ICD	B-NR ACHD 2025
2a	Appropriate ICD therapies for monomorphic VT: adjunctive catheter ablation	B-NR ACHD 2025
2b	Tolerated monomorphic VT from a well-defined isthmus, selected patients: ablation alone instead of ICD at centres with ACHD electrophysiology expertise	C-LD ACHD 2025
IIa	Residual VSD with significant LV volume overload, or during PV surgery: close the VSD	C ESC 2020
IIa	Sustained VT, before surgical or transcatheter valve: catheter mapping and transection of VT isthmuses before or during the procedure	C ESC 2020
IIa	Electrophysiology study for risk stratification with added risk factors (LV/RV dysfunction, symptomatic nonsustained VT, QRS ≥180 ms, extensive RV scar on CMR)	C ESC 2020
IIa	ICD with multiple risk factors (as above, or inducible VT)	C ESC 2020
IIb	Symptomatic monomorphic sustained VT with preserved biventricular function: catheter or surgical ablation instead of ICD in highly experienced centres, once established ablation endpoints are reached (e.g. non-inducibility, conduction block)	C ESC 2020
IIa	Ventricular arrhythmia risk assessment before PVR to select patients for an invasive study with electroanatomic mapping	B-NR STS 2026
IIb	Electrophysiology study with RV electroanatomic mapping before transcatheter or surgical PVR	B-NR STS 2026
I	Arrhythmias fall but do not disappear after PVR: keep reassessing arrhythmia risk	B-NR STS 2026

Branch PA balloon and stent, children (AHA 2011)

Class	Recommendation	Level, source
I	Balloon angioplasty for significant peripheral branch PA stenosis, or PA stenosis in very small patients when a primary stent is not an option	B AHA 2011
IIa	Angioplasty for significant distal stenosis, or larger proximal branch stenosis not suited to a primary stent	B AHA 2011
IIb	Angioplasty for significant main PA stenosis raising proximal PA or RV pressure above 2/3 systemic (without valve stenosis)	C AHA 2011
I	Primary stent for significant proximal or distal branch PA stenosis when the vessel and patient can take a stent dilatable to adult diameter	B AHA 2011
IIa	Stent in critically ill postoperative patients with hemodynamic compromise from branch PA stenosis, in a patient or vessel of any size, particularly if balloon dilation is unsuccessful	B AHA 2011
IIa	Primary stent for significant main PA stenosis raising RV pressure, if it spares a functioning valve and the bifurcation	B AHA 2011
IIb	Small stents without adult-size potential in small children, within a planned surgical strategy	C AHA 2011

Surgical or transcatheter valve?

Favour surgery (AHA 2024 Table 4): other lesions needing surgery (TR, aorta, residual shunt), surgically treatable arrhythmia, RVOT that cannot hold an adequately sized valve or a nonfracturable prosthesis, coronary compression risk, pseudoaneurysm, multilevel obstruction, active endocarditis.
Favour transcatheter: isolated RVOT lesion suited to a transcatheter valve, palliation in children needing frequent interventions, high surgical risk, institutional experience.
Self-expanding valves designed for larger patched RVOTs (Harmony, US approval in early 2022) have substantially expanded TPVR eligibility; experience with them is limited (AHA 2024). In the 2011 pediatric statement, the available valves were for conduits and bioprosthetic valves only.

Key points

Children: echo every 12–24 months; CMR and exercise test from 12 years (AHA 2024; ASE 2014: routine CMR from 10 years).
Symptoms attributable to moderate or greater pulmonary insufficiency: PVR recommended (Class I, B-NR, children and adults, STS 2026).
Adult Moderate PR ≥25% (ACHD 2025 , STS 2026). Without symptoms, criteria differ .

Sources and how this page was checked

Full text read

Geva T, Wald RM, Bucholz E, et al.; AHA Council on Lifelong Congenital Heart Disease and Heart Health in the Young, et al. Long-term management of right ventricular outflow tract dysfunction in repaired tetralogy of Fallot: a scientific statement from the American Heart Association. Circulation 2024;150(25):e689-e707. doi:10.1161/CIR.0000000000001291. Archived publisher copy with Figure 2; expert consensus, no classes. Supplemental table not read.
Valente AM, Cook S, Festa P, et al. Multimodality imaging guidelines for patients with repaired tetralogy of Fallot: a report from the American Society of Echocardiography. J Am Soc Echocardiogr 2014;27(2):111-141. doi:10.1016/j.echo.2013.11.009. ASE-hosted PDF. It gives imaging guidance only, no treatment thresholds.
Nelson JS, Lu JC, Ashfaq A, et al. The Society of Thoracic Surgeons/World Society for Pediatric and Congenital Heart Surgery/European Congenital Heart Surgeons Association 2026 clinical practice guidelines on indications and timing of pulmonary valve replacement in repaired tetralogy of Fallot. Ann Thorac Surg 2026. doi:10.1016/j.athoracsur.2026.04.011. Accepted manuscript (open access, Creative Commons); not compared with the typeset version. ACC/AHA grading system.
Khanna AD, Stephens EH, Jacobs JP, et al. The Society of Thoracic Surgeons (STS)/World Society for Pediatric and Congenital Heart Surgery (WSPCHS)/European Congenital Heart Surgeons Association (ECHSA) expert opinion on the role of exercise testing in determining optimal timing of pulmonary valve replacement in tetralogy of Fallot. Ann Thorac Surg 2026. doi:10.1016/j.athoracsur.2026.05.010. Open access copy; expert opinion, no classes.
Feltes TF, Bacha E, Beekman RH 3rd, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011;123(22):2607-2652. doi:10.1161/CIR.0b013e31821b1f10. Archived publisher copy.
Gurvitz M, Krieger EV, Fuller S, et al. (Writing Committee). 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease. Circulation 2026;153(8):e115-e251 (also J Am Coll Cardiol, 10.1016/j.jacc.2025.09.006). doi:10.1161/CIR.0000000000001402. Archived publisher copy with the recommendation-table and Figure 6 images; replaces the 2018 guideline. Its correction concerns the byline only.
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42(6):563-645. doi:10.1093/eurheartj/ehaa554. Archived publisher copy with the recommendation-table images.

Abstract only

Stephens EH, Zampi JD, Sarris GE, et al. The Society of Thoracic Surgeons / World Society for Pediatric and Congenital Heart Surgery / European Congenital Heart Surgeons Association 2026 expert consensus document on timing, indications, and options for pulmonary valve replacement in children with repaired tetralogy of Fallot. Ann Thorac Surg 2026. doi:10.1016/j.athoracsur.2026.07.010. The abstract reports 13 consensus statements but not their content; none is shown.
Bokma JP, de Wilde KC, Vliegen HW, et al. Value of cardiovascular magnetic resonance imaging in noninvasive risk stratification in tetralogy of Fallot. JAMA Cardiol 2017;2(6):678-683. doi:10.1001/jamacardio.2016.5818. Cohort of the Netherlands score in AHA 2024 Table 2.
Atallah J, Gonzalez Corcia MC, Walsh EP; Pediatric and Congenital Electrophysiology Society. Ventricular arrhythmia and life-threatening events in patients with repaired tetralogy of Fallot. Am J Cardiol 2020;132:126-132. doi:10.1016/j.amjcard.2020.07.012. Cohort of the Congenital Electrophysiology Society score in AHA 2024 Table 2.

How it was checked

Numbers, signs, units and classes are copied from the sources (October 2026); wording is condensed. Classes appear as each source prints them: ACHD 2025 uses 1, 2a, 2b; ESC 2020, STS 2026 and AHA 2011 use I, IIa, IIb. Volumes are written mL/m² throughout. AHA 2024 and ASE 2014 give no classes. Terms follow each source: RVESVi and RVEDVi (ACHD 2025, ESC 2020), RVESV and RVEDV (STS 2026, indexed), “pulmonary insufficiency” (STS 2026), TPVI (ESC 2020), percutaneous PVR (AHA 2011).
All 16 ACHD 2025, 8 ESC 2020 and 6 STS 2026 rows on repaired tetralogy, and the 8 AHA 2011 rows on transcatheter valves and branch PA intervention, are shown. ACHD 2025 conduit recommendations defer to its tetralogy section and are not repeated.
As printed: ACHD 2025 defines moderate PR as ≥25% (Figure 6), >25% (recommendation footnote) and “at least 25%” (text), RVSP as >2/3 (footnotes) and “at least two-thirds” (text); its text calls the 2a progressive-dysfunction row “recommended” and the 2b TR row “should be considered”. AHA 2024 Table 3 and its text give different adult CMR intervals; the statement describes the ESC 2020 pre-PVR electrophysiology advice as “IIB”, while the ESC table prints mapping and isthmus transection in sustained VT as IIa, C. ACHD 2025 Table 32 prints QRS ≥180 ms, its text >180 ms. ASE 2014 Table 6 prints the lung-perfusion criterion “60% systemic” without a sign. Branch PA criteria differ: ≤30% flow (AHA 2024), <35% (ACHD 2025, isolated stenosis), 35%/65% (AHA 2011).
Echo cut-offs quoted from ASE 2014 for RV size, function and RA area come from adult guidelines; pediatric normal values were not available to it.