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cardio.arvc.chronic.v1PRODUCTION
cardio.arvc.chronic.v1

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

cardiologychronicadult
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Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Arrhythmogenic CM suspected (not idiopathic RVOT-VT/athlete heart); classic RV vs ALVC/biventricular

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arrhythmogenic CM phenotype suspected

Patient inputs (10)

Young/athlete SCD risk; surveillance cadence

Endurance/competitive exercise accelerates disease + arrhythmia

RV dilatation/dysfunction/aneurysm — Task Force + risk model input

TWI V1-3, epsilon, TAD — Task Force depolarisation/repolarisation criteria

PVC count + NSVT/VT on Holter — risk model input

Arrhythmic syncope = major ICD risk factor

Prior sustained VT/VF/aborted SCD = secondary-prevention ICD (Class I)

AAD + HF drug dosing; CMR contrast

LV LGE/dysfunction → ALVC/biventricular phenotype + HF therapy

PKP2 vs DSP/PLN/FLNC/TMEM43 — TMEM43/PLN/FLNC malignant → lower ICD threshold

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Severity triggers (9)

9 need judgement
  • informationallife_threateningsustained_va_secondary_prevention
    Prior sustained VT/VF or aborted SCD — secondary-prevention ICD Class I — 2019 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningend_stage_rv_failure
    End-stage RV/biventricular failure or intractable VT despite therapy — transplant evaluation — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehigh_primary_prevention_risk
    Arrhythmic syncope / NSVT / significant RV or LV dysfunction / high Cadrin-Tourigny score — primary-prevention ICD consideration — 2019 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremalignant_genotype_branch
    TMEM43 / PLN / FLNC / DSP variant — malignant arrhythmic phenotype; lower ICD threshold even with modest phenotype — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereathlete_exercise_branch
    Athlete / high-intensity endurance exposure — strict exercise restriction (disease-modifying), shared-decision sports counseling — 2019 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with ARVC — continue beta-blocker, ICD compatible, intensified arrhythmia monitoring; multidisciplinary — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderategene_positive_phenotype_negative
    Pathogenic-variant carrier without phenotype — exercise restriction + serial surveillance (echo/ECG/Holter); not yet ICD — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatealvc_biventricular_branch
    LV-dominant / biventricular (ALVC) phenotype — add HF GDMT (arrhythmia/ICD still dominate) — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateconcomitant_af_branch
    Atrial arrhythmia in ARVC — rate/rhythm + anticoagulation by CHA₂DS₂-VASc — ESC 2024 AF
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

ARVC SCD-prevention + VA management (2019 HRS Arrhythmogenic CM; 2023 ESC Cardiomyopathy)
axis: arvc_scd_prevention_and_vastep 1 - Step 1 — Exercise restriction (cornerstone — ALL patients incl. gene-positive phenotype-negative carriers)
Selected step "Step 1 — Exercise restriction (cornerstone — ALL patients incl. gene-positive phenotype-negative carriers)" — Any confirmed ARVC or pathogenic-variant carrier
  • exercise restriction (no competitive / high-intensity endurance exercise)
    first line
    lifestyle
    triggers: confirmed_ARVC_or_pathogenic_carrier
    2019 HRS — endurance/competitive exercise accelerates structural progression + arrhythmia and increases SCD; restriction is disease-modifying even in phenotype-negative carriers

outpatient playbook — drug actions (4)

  1. 1. exercise restriction counseling
    n/a • n/a • lifelong
    trigger: Confirmed ARVC or pathogenic carrier (2019 HRS)
    Disease-modifying — reduces progression + SCD
  2. 2. beta-blocker
    metoprolol 25 mg BID / bisoprolol 2.5 mg • PO • BID/daily
    trigger: Confirmed ARVC (2019 HRS)
    First-line antiarrhythmic for all
  3. 3. amiodarone/sotalol/flecainide ± ablation
    per agent • PO • per agent
    trigger: Recurrent VA on BB (2019 HRS)
    VA suppression; ablation palliative
  4. 4. risk-based ICD ± HF GDMT
    device / per HFrEF • device/PO • n/a
    trigger: Prior VA or high primary-prevention risk; LV involvement (2019 HRS)
    SCD prevention; GDMT for ALVC

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Echo/CMR: RV dilatation/dysfunction/regional aneurysm (± LV LGE); ECG: T-wave inversion V1–V3, epsilon wave, terminal activation delay; LBBB-morphology VT / frequent PVCs / palpitations.

Objective

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

Assessment

**Arrhythmogenic RV / biventricular cardiomyopathy (ARVC/ALVC, chronic)** (cardio.arvc.chronic.v1).
Phenotype framing: ARVC vs idiopathic RVOT-VT vs cardiac sarcoid vs DCM vs athlete heart vs Brugada
Scope: Arrhythmogenic CM suspected (not idiopathic RVOT-VT/athlete heart); classic RV vs ALVC/biventricular

No severity triggers fired against current inputs.

Plan

Regimen axis: **ARVC SCD-prevention + VA management (2019 HRS Arrhythmogenic CM; 2023 ESC Cardiomyopathy)** — step "Step 1 — Exercise restriction (cornerstone — ALL patients incl. gene-positive phenotype-negative carriers)".
1. exercise restriction (no competitive / high-intensity endurance exercise) (lifestyle, first line) — 2019 HRS — endurance/competitive exercise accelerates structural progression + arrhythmia and increases SCD; restriction is disease-modifying even in phenotype-negative carriers

Setting playbook (outpatient) — Confirm/stage ARVC, exercise-restrict (incl. carriers), risk-stratify for ICD, suppress VA, cascade-screen family (2019 HRS; 2023 ESC Cardiomyopathy)
2. exercise restriction counseling n/a n/a lifelong — Confirmed ARVC or pathogenic carrier (2019 HRS) (Disease-modifying — reduces progression + SCD)
3. beta-blocker metoprolol 25 mg BID / bisoprolol 2.5 mg PO BID/daily — Confirmed ARVC (2019 HRS) (First-line antiarrhythmic for all)
4. amiodarone/sotalol/flecainide ± ablation per agent PO per agent — Recurrent VA on BB (2019 HRS) (VA suppression; ablation palliative)
5. risk-based ICD ± HF GDMT device / per HFrEF device/PO n/a — Prior VA or high primary-prevention risk; LV involvement (2019 HRS) (SCD prevention; GDMT for ALVC)

Non-pharmacologic actions:
- Inherited-cardiomyopathy centre + EP referral — 2019 HRS
- Genetic counseling + first-degree family cascade screening + serial carrier evaluation — 2023 ESC Cardiomyopathy
- Lifelong exercise-restriction reinforcement — 2019 HRS

AVOID / contraindication checks:
- Exercise restriction is disease modifying applies even to gene positive phenotype negative — 2019 HRS
- Ablation is palliative not a substitute for risk based ICD — 2019 HRS
- Lower ICD threshold for TMEM43 PLN FLNC DSP malignant genotypes — 2023 ESC Cardiomyopathy
- Flecainide only with beta blocker and structural caution — 2019 HRS

Monitoring

Regimen monitoring:
- serial echo or CMR q1-2y for RV LV progression — 2019 HRS
- Holter q1-2y for VA burden — 2019 HRS
- ICD interrogation per schedule — 2019 HRS
- amiodarone organ toxicity surveillance TFT LFT PFT — 2019 HRS
- family cascade screening and serial evaluation of carriers — 2023 ESC Cardiomyopathy

Setting (outpatient) monitoring:
- Serial imaging + Holter + ICD checks — 2019 HRS
- Amiodarone organ-toxicity surveillance if used — 2019 HRS

Follow-up plan: Family cascade screening; genotype-specific surveillance of at-risk relatives; lifelong exercise counseling
- Close-out criterion: cascade + long-term plan documented

Monitoring phase: Serial echo/CMR + Holter; ICD interrogation; symptom + VA-burden surveillance

Disposition

Current setting: outpatient — Confirm/stage ARVC, exercise-restrict (incl. carriers), risk-stratify for ICD, suppress VA, cascade-screen family (2019 HRS; 2023 ESC Cardiomyopathy)

Disposition criteria:
- Confirmed ARVC → exercise restriction + BB + risk-based ICD decision + cascade
- Gene-positive phenotype-negative → surveillance + exercise restriction
- End-stage → transplant evaluation

Escalation triggers (move to higher acuity):
- Sustained VT/VF / arrhythmic syncope → EP + ICD now — 2019 HRS
- Progressive RV/biventricular failure → advanced HF / transplant — 2023 ESC Cardiomyopathy
- Electrical storm → emergency (post-arrest ARVC pathway) — 2019 HRS

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Prior sustained VT/VF or aborted SCD — secondary-prevention ICD Class I — 2019 HRS
- [LIFE_THREATENING] End-stage RV/biventricular failure or intractable VT despite therapy — transplant evaluation — 2023 ESC Cardiomyopathy
- [SEVERE] Arrhythmic syncope / NSVT / significant RV or LV dysfunction / high Cadrin-Tourigny score — primary-prevention ICD consideration — 2019 HRS

Citations

- 2019 HRS Arrhythmogenic Cardiomyopathy Expert Consensus + 2023 ESC Cardiomyopathy Guideline; 2010 modified Task Force Criteria [PMID:31170536](https://pubmed.ncbi.nlm.nih.gov/31170536/)
- Cited evidence (PMID 31114936) [PMID:31114936](https://pubmed.ncbi.nlm.nih.gov/31114936/)
- Cited evidence (PMID 20172911) [PMID:20172911](https://pubmed.ncbi.nlm.nih.gov/20172911/)
- Cited evidence (PMID 37622657) [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- Cited evidence (PMID 35379504) [PMID:35379504](https://pubmed.ncbi.nlm.nih.gov/35379504/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2019 HRS Arrhythmogenic Cardiomyopathy Expert Consensus + 2023 ESC Cardiomyopathy Guideline; 2010 modified Task Force Criteria — PMID:31170536
  • Cited evidence (PMID 31114936) — PMID:31114936
  • Cited evidence (PMID 20172911) — PMID:20172911
  • Cited evidence (PMID 37622657) — PMID:37622657
  • Cited evidence (PMID 35379504) — PMID:35379504