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cardio.cpvt.chronic.v1PRODUCTION
cardio.cpvt.chronic.v1

Catecholaminergic polymorphic VT (CPVT, chronic)

cardiologychronicadultpediatric
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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

Confirm CPVT (exercise bidirectional/polymorphic VT, normal resting ECG/QT, structurally normal heart) — not LQTS/ARVC

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Advance rule
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CPVT phenotype framed

Patient inputs (10)

Pediatric common presentation; school AED planning

Competitive/intense exercise = catecholamine trigger to restrict

Normal resting ECG/QT distinguishes CPVT from LQTS

Reproducible exercise bidirectional/polymorphic VT — diagnosis + therapy-response gauge

Exertional/emotional trigger pattern supports CPVT + risk

Aborted SCD = secondary-prevention ICD (with medical therapy)

Flecainide/BB dosing

RYR2 (AD) vs CASQ2 (AR) — cascade + inheritance

Continue BB in pregnancy; peripartum catecholamine surge

Events on BB ± flecainide → LCSD escalation

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Severity triggers (9)

9 need judgement
  • informationallife_threateningaborted_scd_icd_with_medical
    Aborted SCD / sustained VT — secondary-prevention ICD but ALWAYS combined with BB+flecainide±LCSD (ICD shocks pro-arrhythmic in CPVT) — 2022 ESC VA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningicd_shock_storm_branch
    ICD shock storm in CPVT — shocks are pro-arrhythmic (catecholamine surge); intensify BB/flecainide + sedation + LCSD, NOT more shocks alone — 2022 ESC VA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverebb_flecainide_breakthrough_lcsd
    Events / residual exercise VT despite adequate BB + flecainide — LCSD — 2022 ESC VA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereexercise_restriction_branch
    Competitive/intense exercise exposure — primary catecholamine trigger; restriction (shared-decision recreational) — 2022 ESC VA
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepediatric_branch
    Pediatric CPVT — common presentation; weight-based nadolol/flecainide, school AED + emergency action plan — 2013 consensus
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with CPVT — continue nadolol (± flecainide), peripartum catecholamine-surge vigilance; cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateryr2_casq2_genotype_branch
    RYR2 (AD) vs CASQ2 (AR) — inheritance + cascade strategy differ; cascade with exercise test + genetics — 2013 consensus
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderategene_positive_phenotype_negative
    RYR2/CASQ2 carrier without phenotype — nadolol + exercise restriction + serial exercise-test surveillance — 2013 consensus
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — flecainide/BB renal dose-gating — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

CPVT arrhythmia-suppression (2013 HRS/EHRA/APHRS; 2017 AHA/ACC/HRS VA; 2022 ESC VA)
axis: cpvt_arrhythmia_suppressionstep 1 - Step 1 — Nadolol (preferred non-selective BB) — ALL CPVT incl. gene-positive
Selected step "Step 1 — Nadolol (preferred non-selective BB) — ALL CPVT incl. gene-positive" — Confirmed CPVT or RYR2/CASQ2 pathogenic carrier
  • nadolol
    first line
    nonselective_beta_blocker
    1–2 mg/kg/day (typical adult 40–80 mg) • PO • once–BID (max: titrate to exercise-VT suppression/tolerance)
    triggers: confirmed_CPVT_or_pathogenic_carrier
    2022 ESC VA — nadolol is the preferred BB in CPVT (best exercise-VT suppression); lifelong, including gene-positive
    rxcui 7226
  • propranolol
    first line
    nonselective_beta_blocker
    2–3 mg/kg/day • PO • TID
    triggers: nadolol_unavailable
    Propranolol alternative non-selective BB (2017 AHA/ACC/HRS VA)
    rxcui 82084

outpatient playbook — drug actions (3)

  1. 1. nadolol
    1–2 mg/kg/day (40–80 mg) • PO • once–BID
    trigger: Confirmed CPVT / gene+ (2022 ESC VA)
    Preferred BB — first-line for all
  2. 2. flecainide add-on
    100 mg BID • PO • BID
    trigger: Residual exercise VT on BB (van der Werf)
    RyR2 + Na effect — strong adjunct
  3. 3. LCSD / cautious ICD
    procedure / device • surgical/device • n/a
    trigger: Breakthrough / aborted SCD (2022 ESC VA)
    ICD always WITH medical therapy (shocks pro-arrhythmic)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Exertional / emotional syncope with structurally normal heart; Bidirectional / polymorphic VT on exercise stress test; Aborted SCD in a young patient with normal heart/QT.

Objective

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

Assessment

**Catecholaminergic polymorphic VT (CPVT, chronic)** (cardio.cpvt.chronic.v1).
Phenotype framing: CPVT vs LQTS vs ARVC vs idiopathic VT vs Andersen-Tawil
Scope: Confirm CPVT (exercise bidirectional/polymorphic VT, normal resting ECG/QT, structurally normal heart) — not LQTS/ARVC

No severity triggers fired against current inputs.

Plan

Regimen axis: **CPVT arrhythmia-suppression (2013 HRS/EHRA/APHRS; 2017 AHA/ACC/HRS VA; 2022 ESC VA)** — step "Step 1 — Nadolol (preferred non-selective BB) — ALL CPVT incl. gene-positive".
1. nadolol 1–2 mg/kg/day (typical adult 40–80 mg) PO once–BID (nonselective_beta_blocker, first line) — 2022 ESC VA — nadolol is the preferred BB in CPVT (best exercise-VT suppression); lifelong, including gene-positive
2. propranolol 2–3 mg/kg/day PO TID (nonselective_beta_blocker, first line) — Propranolol alternative non-selective BB (2017 AHA/ACC/HRS VA)

Setting playbook (outpatient) — Nadolol all (incl. gene+), flecainide add-on, LCSD for breakthrough, cautious ICD policy, exercise restriction, cascade (2017 AHA/ACC/HRS VA; 2022 ESC VA)
3. nadolol 1–2 mg/kg/day (40–80 mg) PO once–BID — Confirmed CPVT / gene+ (2022 ESC VA) (Preferred BB — first-line for all)
4. flecainide add-on 100 mg BID PO BID — Residual exercise VT on BB (van der Werf) (RyR2 + Na effect — strong adjunct)
5. LCSD / cautious ICD procedure / device surgical/device n/a — Breakthrough / aborted SCD (2022 ESC VA) (ICD always WITH medical therapy (shocks pro-arrhythmic))

Non-pharmacologic actions:
- Inherited-arrhythmia centre + EP referral — 2013 consensus
- Genetic counseling + first-degree family cascade (exercise test + genetics) — 2013 consensus
- Exercise restriction counseling (competitive/intense) — 2022 ESC VA

AVOID / contraindication checks:
- ICD shocks pro arrhythmic in CPVT never standalone always with BB flecainide LCSD — 2022 ESC VA
- Nadolol preferred do not stop even if asymptomatic gene positive — 2022 ESC VA
- Avoid isoproterenol and catecholaminergic agents — 2013 consensus
- Exercise restriction competitive intense exertion — 2022 ESC VA

Monitoring

Regimen monitoring:
- repeat exercise test on therapy for VT suppression — 2022 ESC VA
- beta blocker and flecainide dose adequacy adherence — 2017 AHA/ACC/HRS VA
- ICD interrogation if implanted watch for shock storm — 2022 ESC VA
- family cascade exercise test plus genetics — 2013 consensus

Setting (outpatient) monitoring:
- Repeat exercise test on therapy; BB/flecainide adherence — 2022 ESC VA
- ICD interrogation (shock-storm vigilance) — 2022 ESC VA

Follow-up plan: First-degree family cascade (exercise test + genetics); lifelong BB + exercise counseling
- Close-out criterion: cascade + long-term plan documented

Monitoring phase: Repeat exercise test on therapy (suppression of bidirectional VT), ICD interrogation

Disposition

Current setting: outpatient — Nadolol all (incl. gene+), flecainide add-on, LCSD for breakthrough, cautious ICD policy, exercise restriction, cascade (2017 AHA/ACC/HRS VA; 2022 ESC VA)

Disposition criteria:
- Confirmed CPVT → nadolol + flecainide + exercise restriction + cascade
- Aborted SCD → ICD WITH BB+flecainide±LCSD
- Gene+ phenotype− → nadolol + surveillance + cascade

Escalation triggers (move to higher acuity):
- VT storm / arrest → ED + acute CPVT pathway — 2022 ESC VA
- Breakthrough on BB+flecainide → LCSD — 2022 ESC VA
- ICD shock storm → intensify BB/flecainide/LCSD (NOT just more shocks) — 2022 ESC VA

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Aborted SCD / sustained VT — secondary-prevention ICD but ALWAYS combined with BB+flecainide±LCSD (ICD shocks pro-arrhythmic in CPVT) — 2022 ESC VA
- [LIFE_THREATENING] ICD shock storm in CPVT — shocks are pro-arrhythmic (catecholamine surge); intensify BB/flecainide + sedation + LCSD, NOT more shocks alone — 2022 ESC VA
- [SEVERE] Events / residual exercise VT despite adequate BB + flecainide — LCSD — 2022 ESC VA

Citations

- 2013 HRS/EHRA/APHRS Inherited Arrhythmia Expert Consensus + 2017 AHA/ACC/HRS VA Guideline + 2022 ESC Ventricular Arrhythmia Guideline [PMID:23994779](https://pubmed.ncbi.nlm.nih.gov/23994779/)
- Cited evidence (PMID 29084731) [PMID:29084731](https://pubmed.ncbi.nlm.nih.gov/29084731/)
- Cited evidence (PMID 36017572) [PMID:36017572](https://pubmed.ncbi.nlm.nih.gov/36017572/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2013 HRS/EHRA/APHRS Inherited Arrhythmia Expert Consensus + 2017 AHA/ACC/HRS VA Guideline + 2022 ESC Ventricular Arrhythmia Guideline — PMID:23994779
  • Cited evidence (PMID 29084731) — PMID:29084731
  • Cited evidence (PMID 36017572) — PMID:36017572