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cardio.cpvt.chronic.v1

Catecholaminergic polymorphic VT (CPVT, chronic)

cardiologychronicadultpediatricoutpatienttransition
Start encounter →Print handoutPRODUCTION

CPVT chronic — nadolol lifelong for ALL (incl. gene+), flecainide add-on (RyR2+Na), LCSD for breakthrough, ICD for secondary prevention ALWAYS with BB+flecainide±LCSD (shocks pro-arrhythmic in CPVT), exercise restriction + genetic cascade (exercise test + genetics for relatives). Manifest points at existing sibling cardio.post-arrest.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (arrhythmia-suppression axis + workups + calculators + panels), test_files, 3-PMID evidence object, chronic phases all present. Channelopathy drug RxCUIs RxNav-validated 2026-05-16 (nadolol 7226, propranolol 82084, flecainide 42686); ICD/LCSD + exercise-restriction non_pharm; SNOMED deferred. 9 trigger/special-pop branches: aborted-SCD ICD-with-medical, BB+flecainide breakthrough LCSD, ICD shock-storm, RYR2/CASQ2 genotype, exercise restriction, pediatric, pregnancy, gene+ phenotype−, CKD.

Entry points (5)

  • symptom
    Exertional / emotional syncope with structurally normal heart
    exertional_emotional_syncope
  • imaging
    Bidirectional / polymorphic VT on exercise stress test
    bidirectional_vt_ett
  • history
    Aborted SCD in a young patient with normal heart/QT
    aborted_scd_young
  • history
    Family history of CPVT / exertional young SCD
    family_cpvt_or_scd
  • history
    Known RYR2 / CASQ2 pathogenic variant
    ryr2_casq2_variant

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    Pediatric common presentation; school AED planning
  • exercise_test_resultrequired
    imaging • used at INITIAL_WORKUP
    Reproducible exercise bidirectional/polymorphic VT — diagnosis + therapy-response gauge
  • resting_ecg_qtrequired
    imaging • used at FRAME
    Normal resting ECG/QT distinguishes CPVT from LQTS
  • cpvt_genotype
    history • used at BRANCHING_WORKUP
    RYR2 (AD) vs CASQ2 (AR) — cascade + inheritance
  • syncope_triggerrequired
    history • used at RISK_STRATIFICATION
    Exertional/emotional trigger pattern supports CPVT + risk
  • aborted_scdrequired
    history • used at RISK_STRATIFICATION
    Aborted SCD = secondary-prevention ICD (with medical therapy)
  • exercise_exposurerequired
    history • used at CONTEXT
    Competitive/intense exercise = catecholamine trigger to restrict
  • bb_flecainide_breakthrough
    history • used at RISK_STRATIFICATION
    Events on BB ± flecainide → LCSD escalation
  • creatininerequired
    lab • used at TREATMENT
    Flecainide/BB dosing
  • pregnancy_status
    demographic • used at CONTEXT
    Continue BB in pregnancy; peripartum catecholamine surge

12-phase flow (12)

  1. 1FRAME
    Confirm CPVT (exercise bidirectional/polymorphic VT, normal resting ECG/QT, structurally normal heart) — not LQTS/ARVC
    inputs: resting_ecg_qt, exercise_test_result
    advance: CPVT phenotype framed
  2. 2ENTRY
    Exertional/emotional syncope, bidirectional VT on ETT, aborted SCD, family, gene-positive
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Genotype, exercise exposure, pregnancy
    inputs: exercise_exposure, pregnancy_status
    advance: genetic + exercise context complete
  4. 4RED_FLAGS
    Aborted SCD, VT storm, ICD shock storm (pro-arrhythmic in CPVT)
    inputs: aborted_scd, bb_flecainide_breakthrough
    actions: cardiogenic_shock
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Resting ECG (normal), exercise stress test (reproduce bidirectional VT), Holter
    inputs: exercise_test_result, resting_ecg_qt
    actions: panel.cardiac
    advance: exercise arrhythmia characterised
  6. 6BRANCHING_WORKUP
    Genetic testing (RYR2/CASQ2), epinephrine challenge if needed, family cascade (relatives: exercise test + genetics)
    inputs: cpvt_genotype
    actions: preop_cardiac
    advance: diagnosis + genotype + cascade plan set
  7. 7DIFFERENTIAL
    CPVT vs LQTS vs ARVC vs idiopathic VT vs Andersen-Tawil
    inputs: resting_ecg_qt, exercise_test_result
    advance: CPVT confirmed
  8. 8RISK_STRATIFICATION
    Symptoms/aborted SCD, residual inducible VT on therapy, genotype
    inputs: syncope_trigger, aborted_scd, bb_flecainide_breakthrough
    advance: risk + escalation decision assigned
  9. 9TREATMENT
    Nadolol all (incl. gene+) + flecainide add-on + LCSD for breakthrough + ICD for 2ndary prevention ALWAYS with BB+flecainide±LCSD (shocks pro-arrhythmic) + exercise restriction + cascade
    inputs: creatinine
    advance: nadolol + flecainide + escalation + ICD-policy + cascade plan documented
  10. 10DISPOSITION
    Inherited-arrhythmia centre + EP; genetic counseling; school/sports planning
    inputs: cpvt_genotype
    actions: preop_cardiac
    advance: specialist referral + counseling plan set
  11. 11MONITORING
    Repeat exercise test on therapy (suppression of bidirectional VT), ICD interrogation
    inputs: exercise_test_result
    actions: panel.cardiac
    advance: monitoring cadence documented
  12. 12FOLLOWUP
    First-degree family cascade (exercise test + genetics); lifelong BB + exercise counseling
    inputs: cpvt_genotype
    advance: cascade + long-term plan documented