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cardio.arvc.chronic.v1

Arrhythmogenic RV / biventricular cardiomyopathy (ARVC/ALVC, chronic)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

ARVC/ALVC chronic — SCD-prevention-first: exercise restriction (cornerstone, incl. gene-positive phenotype-negative carriers), risk-stratified ICD (Cadrin-Tourigny + 2019 HRS), BB/AAD/ablation for VA, HF GDMT for ALVC, genetic cascade. Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (SCD-prevention + VA axis + workups + calculators + panels), test_files, 9-PMID evidence object, chronic phases all present. INTEGRATED (not PRODUCTION): BB/AAD/HF RxCUIs reused from validated cardio dossiers; ICD/ablation/transplant/exercise-restriction non_pharm; SNOMED deferred. 9 trigger/special-pop branches: sustained-VA secondary prevention, high primary-prevention risk, malignant genotype, athlete, gene+phenotype−, ALVC, pregnancy, end-stage RV failure, concomitant AF.

Entry points (5)

  • imaging
    Echo/CMR: RV dilatation/dysfunction/regional aneurysm (± LV LGE)
    rv_dysfunction_aneurysm
  • lab_abnormality
    ECG: T-wave inversion V1–V3, epsilon wave, terminal activation delay
    twi_v1_v3_epsilon
  • symptom
    LBBB-morphology VT / frequent PVCs / palpitations
    lbbb_vt_or_pvcs
  • symptom
    Exertional syncope or aborted SCD
    exertional_syncope
  • history
    Family history of SCD / known ARVC desmosomal variant
    family_scd_or_gene

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    Young/athlete SCD risk; surveillance cadence
  • rv_functionrequired
    imaging • used at INITIAL_WORKUP
    RV dilatation/dysfunction/aneurysm — Task Force + risk model input
  • lv_involvement
    imaging • used at BRANCHING_WORKUP
    LV LGE/dysfunction → ALVC/biventricular phenotype + HF therapy
  • ecg_findingsrequired
    imaging • used at INITIAL_WORKUP
    TWI V1-3, epsilon, TAD — Task Force depolarisation/repolarisation criteria
  • ambulatory_va_burdenrequired
    imaging • used at RISK_STRATIFICATION
    PVC count + NSVT/VT on Holter — risk model input
  • arvc_genotype
    history • used at BRANCHING_WORKUP
    PKP2 vs DSP/PLN/FLNC/TMEM43 — TMEM43/PLN/FLNC malignant → lower ICD threshold
  • syncope_historyrequired
    history • used at RISK_STRATIFICATION
    Arrhythmic syncope = major ICD risk factor
  • exercise_exposurerequired
    history • used at CONTEXT
    Endurance/competitive exercise accelerates disease + arrhythmia
  • prior_sustained_varequired
    history • used at RISK_STRATIFICATION
    Prior sustained VT/VF/aborted SCD = secondary-prevention ICD (Class I)
  • creatininerequired
    lab • used at TREATMENT
    AAD + HF drug dosing; CMR contrast

12-phase flow (12)

  1. 1FRAME
    Arrhythmogenic CM suspected (not idiopathic RVOT-VT/athlete heart); classic RV vs ALVC/biventricular
    inputs: rv_function, ecg_findings
    advance: arrhythmogenic CM phenotype suspected
  2. 2ENTRY
    LBBB-VT/PVCs, syncope, TWI V1-3, family SCD, gene-positive
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Genotype, athletic history, family history, exercise exposure
    inputs: exercise_exposure, family_scd_or_gene
    advance: genetic + exercise context complete
  4. 4RED_FLAGS
    Sustained VT/VF, arrhythmic syncope, aborted SCD
    inputs: prior_sustained_va, syncope_history
    actions: cardiogenic_shock
    advance: no red flags or routed to acute/EP pathway
  5. 5INITIAL_WORKUP
    ECG (TWI/epsilon/TAD), echo (RV), Holter (PVC/NSVT/VT burden), signal-averaged ECG
    inputs: rv_function, ecg_findings, ambulatory_va_burden
    actions: panel.cardiac
    advance: Task Force domains assessed
  6. 6BRANCHING_WORKUP
    CMR with LGE (RV + LV), genetic testing, EP study; exclude sarcoid (FDG-PET) when atypical
    inputs: lv_involvement, arvc_genotype
    actions: preop_cardiac
    advance: diagnosis + phenotype + genotype resolved
  7. 7DIFFERENTIAL
    ARVC vs idiopathic RVOT-VT vs cardiac sarcoid vs DCM vs athlete heart vs Brugada
    inputs: rv_function, ecg_findings
    advance: arrhythmogenic CM confirmed/staged (Task Force/Padua)
  8. 8RISK_STRATIFICATION
    Cadrin-Tourigny ARVC risk model; 2019 HRS ICD criteria (prior VA, syncope, NSVT, RV/LV dysfunction, genotype)
    inputs: ambulatory_va_burden, syncope_history, prior_sustained_va
    advance: VA risk + ICD decision assigned
  9. 9TREATMENT
    Exercise restriction (all, incl. carriers) + beta-blocker first-line + AAD/ablation for VA + risk-based ICD + HF GDMT if LV involved
    inputs: creatinine, prior_sustained_va
    advance: exercise + arrhythmia + ICD + HF plan documented
  10. 10DISPOSITION
    Inherited-cardiomyopathy centre; EP for ICD/ablation; genetic counseling
    inputs: arvc_genotype
    actions: preop_cardiac
    advance: specialist referral + counseling plan set
  11. 11MONITORING
    Serial echo/CMR + Holter; ICD interrogation; symptom + VA-burden surveillance
    inputs: rv_function, ambulatory_va_burden
    actions: panel.cardiac
    advance: monitoring cadence documented
  12. 12FOLLOWUP
    Family cascade screening; genotype-specific surveillance of at-risk relatives; lifelong exercise counseling
    inputs: arvc_genotype
    advance: cascade + long-term plan documented