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cardio.achd-tof-repaired.chronic.v1PRODUCTION
cardio.achd-tof-repaired.chronic.v1

Repaired Tetralogy of Fallot (adult, chronic ACHD)

cardiologychronicadult
Hard-required inputs
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Care setting:

Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Adult rTOF — define residual hemodynamic lesions + arrhythmic substrate

Inputs
1
Actions
0
Advance rule
Set
Advance when

rTOF + residual-lesion map framed

Patient inputs (11)

Repair era/age; surveillance + intervention timing

Transannular patch vs conduit vs valve-sparing — PR severity + reintervention type

Severe PR + RV criteria → PVR

RVEDVi/RVESVi/PR fraction/RVEF — the PVR-timing decision driver

QRS ≥180 ms = SCD risk marker

Symptomatic severe PR = PVR; functional status

Contrast for CMR/cath; drug dosing

Aortic root dilatation/AR surveillance

Residual VSD / branch PA stenosis / RVOT obstruction — transcatheter targets

mWHO risk depends on RV/PR/arrhythmia

IART/VT/sustained arrhythmia = PVR + EP indication

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Severity triggers (9)

9 need judgement
  • informationallife_threateningscd_risk_branch
    QRS ≥180 ms + RV dysfunction, or prior sustained VT/aborted SCD — EP study + ICD consideration — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningadvanced_rv_failure
    End-stage RV/biventricular failure despite PVR/therapy — ACHD advanced-HF / transplant evaluation — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepvr_criteria_met
    Symptomatic severe PR OR asymptomatic severe PR + ≥2 RV/arrhythmia/QRS criteria — PVR indicated before irreversible RV dysfunction — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereiart_atrial_flutter_branch
    Intra-atrial reentrant tachycardia / atrial flutter — ablation + anticoagulation; concomitant cryoablation at PVR — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy in rTOF — mWHO II–III by RV/PR/arrhythmia; severe PR + RV dysfunction higher risk; pre-pregnancy PVR consideration; cardio-obstetric — 2018 AHA/ACC ACHD; ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateaortic_root_branch
    Aortic root dilatation/AR in rTOF — surveillance imaging; surgery at ACHD-specific threshold — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateresidual_lesion_branch
    Residual VSD / branch PA stenosis / RVOT obstruction — transcatheter or surgical correction — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateendocarditis_prophylaxis_branch
    Prosthetic pulmonary valve/conduit — IE prophylaxis before high-risk dental procedures + low threshold for blood cultures with fever — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatetransition_of_care_branch
    Pediatric→adult ACHD transition — structured handoff; loss-to-follow-up is a major preventable risk — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

rTOF — PVR timing + arrhythmia/SCD + residual-lesion management (2018 AHA/ACC ACHD; 2020 ESC ACHD)
axis: rtof_pvr_and_arrhythmiastep 1 - Step 1 — Surveillance + define residual lesions (CMR-driven)
Selected step "Step 1 — Surveillance + define residual lesions (CMR-driven)" — rTOF adult without current PVR/intervention indication

outpatient playbook — drug actions (2)

  1. 1. PVR referral when criteria met
    procedure • surgical/transcatheter • n/a
    trigger: Severe PR + RV criteria (2018 AHA/ACC ACHD)
    Preserve RV function
  2. 2. arrhythmia ablation ± ICD; amiodarone adjunct
    procedure/device; amiodarone load→200 mg • varied/PO • n/a/daily
    trigger: IART/VT or SCD risk (2018 AHA/ACC ACHD)
    Arrhythmia control + SCD prevention

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Pulmonary regurgitation murmur / RV dilatation on imaging; Reduced exercise tolerance / dyspnea in rTOF adult; QRS duration ≥180 ms (SCD risk marker).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Repaired Tetralogy of Fallot (adult, chronic ACHD)** (cardio.achd-tof-repaired.chronic.v1).
Phenotype framing: PR-dominant vs RVOT obstruction vs residual VSD vs branch PA stenosis vs aortic root pathology
Scope: Adult rTOF — define residual hemodynamic lesions + arrhythmic substrate

No severity triggers fired against current inputs.

Plan

Regimen axis: **rTOF — PVR timing + arrhythmia/SCD + residual-lesion management (2018 AHA/ACC ACHD; 2020 ESC ACHD)** — step "Step 1 — Surveillance + define residual lesions (CMR-driven)".

Setting playbook (outpatient) — CMR-guided PVR timing, arrhythmia/SCD risk-stratification, residual-lesion + aortic surveillance, lifelong ACHD care (2018 AHA/ACC ACHD; 2020 ESC ACHD)
1. PVR referral when criteria met procedure surgical/transcatheter n/a — Severe PR + RV criteria (2018 AHA/ACC ACHD) (Preserve RV function)
2. arrhythmia ablation ± ICD; amiodarone adjunct procedure/device; amiodarone load→200 mg varied/PO n/a/daily — IART/VT or SCD risk (2018 AHA/ACC ACHD) (Arrhythmia control + SCD prevention)

Non-pharmacologic actions:
- Lifelong ACHD-centre + EP referral — 2018 AHA/ACC ACHD
- Endocarditis prophylaxis education (prosthetic valve/conduit) — 2018 AHA/ACC ACHD
- Pregnancy + transition-of-care counseling — 2018 AHA/ACC ACHD

AVOID / contraindication checks:
- Replace pulmonary valve before irreversible RV dysfunction RVESVi driven — 2018 AHA/ACC ACHD
- QRS ge 180ms plus RV dysfunction is an SCD risk marker EP evaluation — 2018 AHA/ACC ACHD
- Lifelong ACHD centre surveillance not general cardiology alone — 2018 AHA/ACC ACHD
- Endocarditis prophylaxis for prosthetic valve conduit — 2018 AHA/ACC ACHD

Monitoring

Regimen monitoring:
- serial CMR RV volumes and PR fraction — 2018 AHA/ACC ACHD
- echo and ECG QRS trend — 2018 AHA/ACC ACHD
- Holter and CPET periodic — 2020 ESC ACHD
- post PVR valve function surveillance — 2018 AHA/ACC ACHD
- aortic root imaging periodic — 2018 AHA/ACC ACHD

Setting (outpatient) monitoring:
- Serial CMR/echo/Holter/QRS — 2018 AHA/ACC ACHD

Follow-up plan: Lifelong ACHD follow-up; pregnancy + transition planning; endocarditis education
- Close-out criterion: lifelong ACHD + counseling plan documented

Monitoring phase: Serial CMR (RV volumes), echo, Holter, QRS trend; post-PVR valve surveillance

Disposition

Current setting: outpatient — CMR-guided PVR timing, arrhythmia/SCD risk-stratification, residual-lesion + aortic surveillance, lifelong ACHD care (2018 AHA/ACC ACHD; 2020 ESC ACHD)

Disposition criteria:
- Meets PVR criteria → structural referral
- Arrhythmia/SCD risk → EP + ICD decision
- Stable → ACHD-centre periodic surveillance

Escalation triggers (move to higher acuity):
- RV dysfunction approaching irreversibility → expedite PVR — 2018 AHA/ACC ACHD
- Sustained VT / aborted SCD → EP + ICD — 2018 AHA/ACC ACHD
- Decompensated RV failure → ACHD heart-failure / transplant — 2020 ESC ACHD

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] QRS ≥180 ms + RV dysfunction, or prior sustained VT/aborted SCD — EP study + ICD consideration — 2018 AHA/ACC ACHD
- [LIFE_THREATENING] End-stage RV/biventricular failure despite PVR/therapy — ACHD advanced-HF / transplant evaluation — 2020 ESC ACHD
- [SEVERE] Symptomatic severe PR OR asymptomatic severe PR + ≥2 RV/arrhythmia/QRS criteria — PVR indicated before irreversible RV dysfunction — 2018 AHA/ACC ACHD

Citations

- 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) [PMID:30121239](https://pubmed.ncbi.nlm.nih.gov/30121239/)
- Cited evidence (PMID 32860028) [PMID:32860028](https://pubmed.ncbi.nlm.nih.gov/32860028/)
- Cited evidence (PMID 36017572) [PMID:36017572](https://pubmed.ncbi.nlm.nih.gov/36017572/)
- Cited evidence (PMID 32673028) [PMID:32673028](https://pubmed.ncbi.nlm.nih.gov/32673028/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner) — PMID:30121239
  • Cited evidence (PMID 32860028) — PMID:32860028
  • Cited evidence (PMID 36017572) — PMID:36017572
  • Cited evidence (PMID 32673028) — PMID:32673028