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cardio.achd-tof-repaired.chronic.v1

Repaired Tetralogy of Fallot (adult, chronic ACHD)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

Repaired Tetralogy of Fallot adult — CMR-RV-volume-driven PVR timing + arrhythmia/SCD (QRS≥180 + RV dysfunction) + residual-lesion (VSD/branch-PA) + aortic-root surveillance; lifelong ACHD-centre care + transition. Manifest points at existing sibling cardio.valvular_disease.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (PVR + arrhythmia + residual-lesion axis + workups + calculators + panels), test_files, 4-PMID evidence object, chronic phases all present. INTEGRATED (not PRODUCTION): structural interventions (PVR/ablation/ICD/transcatheter) non_pharm; amiodarone 703 / furosemide 4603 RxCUIs reused from validated cardio dossiers; SNOMED deferred. 9 trigger/special-pop branches: PVR-criteria, SCD-risk, IART/atrial-flutter, aortic-root, residual-lesion, pregnancy, endocarditis-prophylaxis, transition-of-care, advanced RV failure.

Entry points (5)

  • symptom
    Pulmonary regurgitation murmur / RV dilatation on imaging
    pr_murmur_rv_dilatation
  • symptom
    Reduced exercise tolerance / dyspnea in rTOF adult
    exercise_intolerance
  • lab_abnormality
    QRS duration ≥180 ms (SCD risk marker)
    wide_qrs
  • symptom
    Atrial flutter/IART or VT / palpitations
    palpitations_arrhythmia
  • history
    Transition from pediatric to adult ACHD care
    transition_to_adult_achd

Required inputs (11)

  • agerequired
    demographic • used at CONTEXT
    Repair era/age; surveillance + intervention timing
  • repair_detailsrequired
    history • used at CONTEXT
    Transannular patch vs conduit vs valve-sparing — PR severity + reintervention type
  • cmr_rv_volumesrequired
    imaging • used at RISK_STRATIFICATION
    RVEDVi/RVESVi/PR fraction/RVEF — the PVR-timing decision driver
  • pr_severityrequired
    imaging • used at INITIAL_WORKUP
    Severe PR + RV criteria → PVR
  • qrs_durationrequired
    imaging • used at RISK_STRATIFICATION
    QRS ≥180 ms = SCD risk marker
  • sustained_arrhythmia
    history • used at RISK_STRATIFICATION
    IART/VT/sustained arrhythmia = PVR + EP indication
  • aortic_root
    imaging • used at BRANCHING_WORKUP
    Aortic root dilatation/AR surveillance
  • residual_lesions
    imaging • used at BRANCHING_WORKUP
    Residual VSD / branch PA stenosis / RVOT obstruction — transcatheter targets
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Symptomatic severe PR = PVR; functional status
  • creatininerequired
    lab • used at TREATMENT
    Contrast for CMR/cath; drug dosing
  • pregnancy_status
    demographic • used at CONTEXT
    mWHO risk depends on RV/PR/arrhythmia

12-phase flow (12)

  1. 1FRAME
    Adult rTOF — define residual hemodynamic lesions + arrhythmic substrate
    inputs: repair_details
    advance: rTOF + residual-lesion map framed
  2. 2ENTRY
    PR/RV dilatation, exercise intolerance, wide QRS, arrhythmia, transition
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Repair type/era, conduit, residual lesions, prior arrhythmia, pregnancy
    inputs: repair_details, pregnancy_status
    advance: repair + comorbidity context complete
  4. 4RED_FLAGS
    Sustained VT, decompensated RV failure, endocarditis (conduit/prosthesis)
    inputs: sustained_arrhythmia, nyha_class
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo (PR, RV, RVOT), ECG (QRS), NT-proBNP
    inputs: pr_severity, qrs_duration
    actions: panel.cardiac
    advance: baseline PR/RV/QRS documented
  6. 6BRANCHING_WORKUP
    Cardiac MRI (RV volumes/PR fraction/RVEF), Holter, CPET, EP study; aortic root + residual-lesion assessment
    inputs: cmr_rv_volumes, aortic_root, residual_lesions
    actions: preop_cardiac
    advance: RV quantification + arrhythmic + residual-lesion status resolved
  7. 7DIFFERENTIAL
    PR-dominant vs RVOT obstruction vs residual VSD vs branch PA stenosis vs aortic root pathology
    inputs: pr_severity, residual_lesions
    advance: dominant lesion(s) assigned
  8. 8RISK_STRATIFICATION
    PVR criteria (severe PR + RVEDVi >160 mL/m²/RVESVi/RVEF decline/arrhythmia/QRS≥180/exercise); SCD risk
    inputs: cmr_rv_volumes, qrs_duration, sustained_arrhythmia, nyha_class
    advance: PVR + SCD/ICD decisions assigned
  9. 9TREATMENT
    PVR timing (surgical or transcatheter Melody/Sapien); IART/VT ablation + ICD by risk; transcatheter residual-lesion intervention; aortic surveillance ± surgery; endocarditis prophylaxis
    inputs: creatinine
    advance: PVR + arrhythmia + residual-lesion plan documented
  10. 10DISPOSITION
    ACHD centre + EP; structural intervention referral
    inputs: nyha_class
    actions: preop_cardiac
    advance: ACHD-centre referral plan set
  11. 11MONITORING
    Serial CMR (RV volumes), echo, Holter, QRS trend; post-PVR valve surveillance
    inputs: cmr_rv_volumes, qrs_duration
    actions: panel.cardiac
    advance: surveillance cadence documented
  12. 12FOLLOWUP
    Lifelong ACHD follow-up; pregnancy + transition planning; endocarditis education
    inputs: pregnancy_status
    advance: lifelong ACHD + counseling plan documented