Clinical Commander

Back to dossier
cardio.post-arrest.arvc-related.v1PRODUCTION
cardio.post-arrest.arvc-related.v1

Post-cardiac-arrest care — arrhythmogenic right-ventricular cardiomyopathy (ARVC; PKP2 / DSP desmosomal; exercise-induced VT/VF arrest)

cardiologyacuteadultpediatric
Hard-required inputs
0 / 23
Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Recognize post-ROSC + exercise-induced arrest in young patient + characteristic ECG (T-wave inversion V1-V3 beyond age 14, epsilon waves) + RV abnormalities on echo as ARVC-arrest cohort; pivot from generic post-arrest care to channelopathy-specific endurance-exercise avoidance + cardiac MRI scheduling + Padua criteria application; route to parent cardio.post-arrest.core.v1 for TTM + neuroprog while specializing on cardiac MRI + family screening + β-blocker + sotalol + ICD + catheter ablation + LV-dominant management arcs

Inputs
3
Actions
0
Advance rule
Set
Advance when

ARVC high pretest probability confirmed + structural disease screen initiated + cardiac MRI scheduled

Patient inputs (26)

ARVC typically manifests in young adulthood (median age first event ~25-40 y; rare in childhood under age 12 — but cascade family-screening can identify pediatric carriers); pediatric resuscitation modifications below age 8; ICD device-size considerations + endurance-sport counseling are particularly difficult conversations in young athletes

Male predominance ~3:1 — male sex + endurance exercise both worsen phenotype expression even at same genotype; informs cascade-screening prioritization

Trigger pattern is highly informative — exercise trigger (especially endurance training history: cycling, running, swimming, triathlon) is the ARVC signature; informs lifelong endurance-exercise avoidance counseling (THE most disease-modifying intervention); drives genetic panel + cardiac MRI referral

CPR within 1 min dramatically improves outcome; CAHP/OHCA score input

AED within 3 min → 50%+ survival; venue AED program (sports field, gym, race venue) presence is dominant prognostic factor + suggests exercise-trigger context

VF most common terminal rhythm; sustained / non-sustained VT with LBBB superior axis morphology (RV-origin VT) is Padua MAJOR criterion when documented pre-arrest; informs storm-suppression bridge planning + ablation candidacy

Family history of SCD <35 y, known ARVC (autosomal dominant PKP2 in ~30-45%; DSP in ~15-25%; others rare), or familial pattern of exercise-induced arrhythmia is critical input — drives cascade cardiac-MRI + ECG + Holter + exercise-stress + genotyping screening of relatives

Lifetime endurance exercise dose (years of competitive cycling, running, triathlon, swimming) directly correlates with phenotype severity per Saberniak 2014 + James 2013 — quantify hours/week and years; informs disease-modifier counseling

Comprehensive medication review; class I antiarrhythmics generally avoided in structural heart disease (CAST framework — proarrhythmic in scar substrate); document baseline meds

eGFR for sotalol dose adjustment (renally cleared — proarrhythmic in CKD; reduce dose or avoid if eGFR <40); β-blocker also dose-adjusted

Cardiac MRI at 4–6 wk post-arrest GOLD STANDARD diagnostic — RV regional wall-motion abnormalities + RV dilation + RV dysfunction with fibrofatty replacement on T1 / late gadolinium enhancement (Padua MAJOR criterion when meeting thresholds); LV LGE in subepicardial / midwall pattern in left-dominant / biventricular ARVC (DSP, PLN); allows post-arrest stunning to resolve before baseline assessment

Targeted gene panel — PKP2 core (~30-45% positive — autosomal dominant); expanded panel DSP (~15-25%; left-dominant variant common), DSG2 (~7-10%), JUP (~1-5%; Naxos cardiocutaneous syndrome), DSC2 (~1-2%), TMEM43 (~1-5%; founder Newfoundland), PLN (~1-5%; biventricular left-dominant), CDH2, TJP1; per HRS 2019 ARVC genetic statement PMID 31345875; informs prognosis + cascade testing

Serial 12-lead ECG documenting T-wave inversion V1-V3 beyond age 14 (Padua MAJOR), epsilon waves V1-V3 (~30% sensitivity; Padua MAJOR — best seen on Fontaine bipolar leads), prolonged terminal activation duration ≥55 ms (Padua minor), QRS fragmentation; baseline for ICD planning + ablation substrate mapping

STAT echo with RV-focused assessment — RV regional wall-motion abnormality (free-wall aneurysm, akinetic outflow tract), RV dilation (RVOT PLAX ≥32 mm or RVOT PSAX ≥36 mm at end-diastole = Padua MAJOR), RV dysfunction (RVEF reduced); LV size + function for biventricular involvement; structural finding pivots toward ARVC-specific cardiac MRI

Often modestly elevated from arrest + CPR; rise pattern helps differentiate from ACS-mediated arrest; primary ARVC arrest typically negative or modest

Tissue hypoperfusion + post-arrest perfusion debt; clearance trajectory drives prognosis (SCAI 2022 PMID 35718438; CardShock PMID 26333869)

K target ≥4.0 standard (≥4.5 if on sotalol — sotalol is QT-prolonging Class III); replacement to suppress polymorphic VT recurrence

Mg target ≥2.0 standard; replacement to suppress polymorphic VT recurrence

Rib fractures from CPR; pneumothorax; aspiration; baseline for ICD lead placement planning; cardiomegaly assessment

MAP ≥65 target post-ROSC; SCAI staging if shock; cautious vasopressor (minimize epinephrine + dobutamine load on weakened desmosomal junctions); biventricular ARVC may have HF physiology

β-blocker initiation depends on rate; monomorphic VT often LBBB superior axis morphology in ARVC

TTM target 33–37.5 °C × 24 h (TTM2 PMID 34133859); shivering control during rewarm to minimize sympathetic load on weakened desmosomes

Avoid hyperoxia: SpO2 92–98% (AHA 2020 Class IIa)

Signal-averaged ECG (SAECG) — late potentials (filtered QRS duration >114 ms; LAS40 >38 ms; RMS40 <20 µV) is Padua MINOR criterion; useful screening adjunct but cardiac MRI more sensitive

24-48 hr ambulatory monitoring quantifying PVC burden (>500/24 h = Padua MINOR; >1000/24 h higher specificity); non-sustained / sustained VT episodes; informs ICD planning + ablation candidacy

Exercise stress test under EP supervision post-stabilization — quantifies exercise-induced ectopy + non-sustained / sustained VT; informs activity counseling + risk stratification; performed at inherited-arrhythmia center

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (5)

5 need judgement
  • informationallife_threateningrecurrent_vt_post_rosc_arvc_storm_bridge
    Recurrent VT (typically LBBB superior axis morphology = RV-origin) post-ROSC suggests ongoing ARVC storm physiology — esmolol IV bridge → metoprolol/nadolol PO + magnesium + sotalol bridge once stable; AVOID class I antiarrhythmics (flecainide, propafenone — proarrhythmic in scar substrate); refractory storm → urgent transfer to expert ARVC ablation center for combined endo + epicardial substrate ablation
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinadvertent_class_i_antiarrhythmic_administered_post_rosc
    Inadvertent administration of class I antiarrhythmic (flecainide, propafenone) post-ROSC in confirmed or suspected ARVC — STOP drug + escalate to EP + chart audit + nursing handoff review; substitute amiodarone or lidocaine bridge if needed; class I antiarrhythmics are proarrhythmic in scar substrate per CAST framework
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereicd_eligibility_evaluation_arvc_post_arrest_secondary_prevention
    Sustained VT/VF survivor by definition meets HRS 2019 ARVC + ITF 2020 Class I ICD criteria as SECONDARY PREVENTION — implant pre-discharge or schedule within 1 wk; subcutaneous ICD considered if no pacing indication (RV-lead-related issues common with desmosomal disease); WCD bridge if ICD deferred for stabilization
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecascade_family_screening_with_cardiac_mri_referral_required
    Confirmed ARVC (or strongly suspected pending genetic results + cardiac MRI) triggers mandatory cascade screening of first-degree relatives — multimodality screening: ECG + echo + cardiac MRI (critical element — gold-standard diagnostic far superior to ECG alone) + signal-averaged ECG + 24-h Holter + exercise stress test + genetic testing at proband mutation; many newly identified relatives are asymptomatic carriers requiring lifelong surveillance + endurance-exercise counseling
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereendurance_exercise_avoidance_counseling_critical
    LIFELONG ENDURANCE EXERCISE + COMPETITIVE SPORTS AVOIDANCE counseling per HRS 2019 + ITF 2020 Class I — THE most disease-modifying intervention; James 2013 + Saberniak 2014 + Sawant 2014 evidence base — endurance exercise dramatically accelerates fibrofatty replacement and disease progression; dose-response with hours/week and years; difficult conversation in young athletes (identity loss) requires multidisciplinary approach including mental health support; recreational low-intensity activity ≤6 METs titrated to symptoms
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

TREATMENTrequiredDrives monitoring threshold
Loading…

Recommended regimen

ARVC post-arrest phenotype — standard post-ROSC bundle with cautious sympathomimetic minimization + LONG-TERM β-blocker + sotalol for sustained VT or post-ICD-shock + ICD pathway (HRS 2019 ARVC + ITF 2020 Class I as secondary prevention) + catheter ablation for refractory storm at expert ARVC center + LIFELONG endurance-exercise avoidance + cascade family screening with cardiac MRI
axis: arvc_post_arrest_phenotype
Selected axis "ARVC post-arrest phenotype — standard post-ROSC bundle with cautious sympathomimetic minimization + LONG-TERM β-blocker + sotalol for sustained VT or post-ICD-shock + ICD pathway (HRS 2019 ARVC + ITF 2020 Class I as secondary prevention) + catheter ablation for refractory storm at expert ARVC center + LIFELONG endurance-exercise avoidance + cascade family screening with cardiac MRI" by default fallback (first axis)
  • nadolol
    first line
    non_selective_beta_blocker_long_acting
    ARVC long-term: nadolol 1-1.5 mg/kg/d PO daily (alternative to metoprolol succinate; long half-life advantage) • PO • daily; lifelong
    triggers: confirmed_arvc_post_arrest, long_term_arrhythmia_suppression
    HRS 2019 ARVC PMID 31345875 IIa + HRS 2017 PMID 28219760 — β-blocker first-line for arrhythmia suppression in ARVC; nadolol or metoprolol succinate equivalent options
    rxcui 7226
  • metoprolol succinate
    first line
    selective_beta1_blocker_extended_release
    ARVC long-term alternate: metoprolol succinate 25-200 mg PO daily titrated • PO • daily; lifelong
    triggers: confirmed_arvc_post_arrest, long_term_arrhythmia_suppression, lv_dominant_phenotype_with_hf
    HRS 2019 ARVC IIa; preferred in LV-dominant / biventricular phenotype with HF (β1-selective, GDMT pillar)
    rxcui 6918
  • sotalol
    add on
    class_iii_antiarrhythmic_with_beta_blocker
    ARVC sustained VT or post-ICD-shock: sotalol 80-160 mg PO BID (renal dose-adjustment if eGFR <60; AVOID if eGFR <40) • PO • BID
    triggers: sustained_vt_post_arrest_in_arvc, recurrent_icd_shocks_in_arvc
    HRS 2017 IIa + HRS 2019 ARVC IIa — Class III + β-blocker dual mechanism; QTc surveillance mandatory; K ≥4.5 + Mg ≥2.0 mandatory; renal dose-adjustment critical
    rxcui 9947
  • magnesium sulfate
    first line
    electrolyte_membrane_stabilizer
    2 g IV over 5–15 min then 2 g/h infusion supportive for polymorphic VT + shivering suppression during TTM rewarm • IV • continuous
    triggers: polymorphic_vt_post_rosc_supportive, shivering_during_ttm_rewarm, mandatory_target_with_sotalol
    AHA 2020 ACLS supportive for polymorphic VT; mandatory target ≥2.0 with sotalol initiation (QT prolongation safety)
    rxcui 6585
  • potassium chloride
    first line
    electrolyte
    20–40 mEq IV/PO until K ≥4.0 standard (≥4.5 mandatory if on sotalol) • IV/PO • PRN until target sustained
    triggers: k_below_4_post_rosc, k_below_4.5_on_sotalol_mandatory_target
    Standard polymorphic VT supportive therapy; mandatory ≥4.5 with sotalol (Class III QT-prolonging)
    rxcui 8591
  • norepinephrine
    first line
    vasopressor_alpha1_beta1
    0.05–0.5 µg/kg/min titrate MAP ≥65; cautious in ARVC (sympathomimetic load on weakened desmosomes); minimize dose; substitute vasopressin or phenylephrine when feasible • IV • continuous
    triggers: post_rosc_vasoplegia
    SOAP-II PMID 20200382; first-line post-ROSC vasoactive; in ARVC use lowest effective dose to minimize sympathomimetic stress on desmosomal junctions
    rxcui 7512
  • vasopressin
    second line
    vasopressor_v1_agonist
    0.03 U/min IV fixed dose (catecholamine-sparing alternative) • IV • continuous
    triggers: post_rosc_vasoplegia_in_arvc_minimize_sympathomimetic_load
    Non-adrenergic vasopressor preferred adjunct in ARVC to minimize sympathomimetic stress on weakened desmosomes
    rxcui 11149
  • milrinone
    comorbidity specific
    phosphodiesterase_3_inhibitor_inodilator
    0.125–0.5 µg/kg/min IV (catecholamine-sparing inotrope alternative for biventricular ARVC with HF) • IV • continuous
    triggers: post_rosc_inotropic_support_avoiding_dobutamine_in_arvc, biventricular_arvc_hf_decompensation
    PDE3 pathway — non-catecholamine inotrope; preferred substitute for dobutamine in ARVC to minimize sympathomimetic stress; particularly useful in biventricular phenotype with HF
    rxcui 52769
  • epinephrine
    first line
    inotrope_chronotrope_vasopressor
    1 mg IV q3–5 min during arrest only (ACLS standard); minimize post-ROSC infusion if alternative • IV • standard ACLS only
    triggers: cardiac_arrest_during_index_event
    AHA 2020 ACLS — standard arrest pathway; minimize post-ROSC infusion to limit sympathomimetic load
    rxcui 3992
  • propofol
    first line
    sedative_iv_anesthetic
    5–50 µg/kg/min; titrate RASS • IV • continuous
    triggers: post_rosc_intubation_ttm
    PADIS 2018; preferred sedative for post-ROSC TTM (no specific ARVC contraindication unlike Brugada)
    rxcui 8782
  • fentanyl
    first line
    opioid_analgesic
    25–200 µg/h • IV • continuous
    triggers: post_rosc_intubation_ttm, analgesia_ttm
    PADIS 2018; preferred opioid for analgesia + shivering suppression during TTM rewarm
    rxcui 4337
  • dexmedetomidine
    second line
    alpha2_agonist_sedative
    0.2–1.4 µg/kg/h; no bolus • IV • continuous
    triggers: post_rosc_delirium_prevention, shivering_suppression_during_ttm_rewarm
    PADIS 2018; α2 agonist actually REDUCES central sympathetic outflow — useful in ARVC for shivering suppression + delirium prevention
    rxcui 48937
  • acetaminophen
    first line
    antipyretic_analgesic
    650 mg PO/PR/IV q6h PRN • PO/PR/IV • q6h PRN
    triggers: analgesia_avoid_nsaids_in_aki_or_hf, fever_post_arrest
    Standard analgesia / antipyresis; AVOID NSAIDs (fluid retention + RV adverse remodeling concern in ARVC HF)
    rxcui 161
  • lidocaine
    rescue
    class_ib_sodium_channel_blocker
    1–1.5 mg/kg IV bolus then 1–4 mg/min infusion bridge for refractory polymorphic VT during arrest pathway • IV • continuous bridge
    triggers: refractory_polymorphic_vt_during_arrest_no_alternative
    AHA 2020 ACLS standard polymorphic VT bridge; acceptable in ARVC (unlike Brugada where contraindicated); β-blocker + sotalol preferred long-term
    rxcui 6387
  • amiodarone
    rescue
    class_iii_antiarrhythmic
    150-300 mg IV bolus then 1 mg/min × 6 h then 0.5 mg/min × 18 h for refractory VT bridge • IV • continuous bridge
    triggers: refractory_vt_during_acute_post_arrest_no_alternative
    AHA 2020 ACLS for VT/VF; acceptable bridge in ARVC; long-term sotalol preferred per HRS 2019 IIa
    rxcui 703

outpatient playbook — drug actions (4)

  1. 1. continue β-blocker maintenance lifelong
    rxcui 7226
    nadolol 1-1.5 mg/kg/d OR metoprolol succinate 25-200 mg/d • PO • daily; lifelong
    trigger: ARVC diagnosis
    HRS 2019 ARVC IIa
  2. 2. continue sotalol lifelong if on therapy with QTc surveillance
    rxcui 37418
    80-160 mg PO BID (renal dose-adjustment) • PO • BID; lifelong
    trigger: sustained VT or post-ICD-shock burden
    HRS 2019 + HRS 2017 IIa
  3. 3. continue lifelong avoidance of class I antiarrhythmics + endurance exercise + competitive sports
    patient education + curated counseling • n/a • lifelong
    trigger: ARVC diagnosis
    CAST + HRS 2019 + ITF 2020 Class I
  4. 4. GDMT 4-pillar for biventricular / LV-dominant ARVC with HFrEF (ARNI + β-blocker + MRA + SGLT2i)
    per HF guideline-directed • PO • daily; lifelong
    trigger: biventricular / LV-dominant ARVC with HFrEF
    HRS 2019 ARVC IIa + HF guideline directed therapy

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: ROSC after out-of-hospital VF arrest with known ARVC (prior diagnosis, prior ICD, prior syncope, family history) or with sentinel pre-arrest narrative — exercise-induced collapse in young patient (often endurance athlete) with characteristic ECG changes (T-wave inversion V1-V3 beyond age 14, epsilon waves); Post-ROSC 12-lead ECG with T-wave inversion in V1-V3 beyond age 14 (Padua MAJOR criterion) ± epsilon waves V1-V3 (Padua MAJOR criterion; ~30% sensitivity) ± prolonged terminal activation duration ≥55 ms — pivot to ARVC-specific workup including cardiac MRI; Witnessed arrest during exercise (running, cycling, swimming, competitive sports — particularly endurance athletes) in young patient (childhood through young adulthood) — high pretest probability for ARVC; endurance exercise both trigger and disease-modifier (accelerates fibrofatty replacement).

Objective

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

Assessment

**Post-cardiac-arrest care — arrhythmogenic right-ventricular cardiomyopathy (ARVC; PKP2 / DSP desmosomal; exercise-induced VT/VF arrest)** (cardio.post-arrest.arvc-related.v1).
Phenotype framing: ARVC (T-wave inversion V1-V3 beyond age 14 + epsilon waves + RV regional WMA + structurally abnormal heart on cardiac MRI with fibrofatty replacement; exercise trigger; PKP2 / DSP autosomal dominant) vs CPVT (functional channelopathy — calcium-handling RYR2 / CASQ2; NORMAL resting ECG; bidirectional VT on exercise stress test; structurally NORMAL heart) vs Brugada (Type 1 V1-V3 coved ST + sleep / fever trigger; sodium channelopathy) vs sarcoidosis (multifocal LGE on cardiac MRI + extracardiac involvement — granulomas; PET-CT for metabolic activity) vs RVOT idiopathic VT (LBBB inferior axis VT in structurally normal heart — distinct from ARVC LBBB superior axis) vs Uhl anomaly (rare congenital RV dysplasia — distinct phenotype) vs idiopathic VF — this differential drives long-term plan + family screening
Scope: Recognize post-ROSC + exercise-induced arrest in young patient + characteristic ECG (T-wave inversion V1-V3 beyond age 14, epsilon waves) + RV abnormalities on echo as ARVC-arrest cohort; pivot from generic post-arrest care to channelopathy-specific endurance-exercise avoidance + cardiac MRI scheduling + Padua criteria application; route to parent cardio.post-arrest.core.v1 for TTM + neuroprog while specializing on cardiac MRI + family screening + β-blocker + sotalol + ICD + catheter ablation + LV-dominant management arcs

No severity triggers fired against current inputs.

Plan

Regimen axis: **ARVC post-arrest phenotype — standard post-ROSC bundle with cautious sympathomimetic minimization + LONG-TERM β-blocker + sotalol for sustained VT or post-ICD-shock + ICD pathway (HRS 2019 ARVC + ITF 2020 Class I as secondary prevention) + catheter ablation for refractory storm at expert ARVC center + LIFELONG endurance-exercise avoidance + cascade family screening with cardiac MRI**.
1. nadolol ARVC long-term: nadolol 1-1.5 mg/kg/d PO daily (alternative to metoprolol succinate; long half-life advantage) PO daily; lifelong (non_selective_beta_blocker_long_acting, first line) — HRS 2019 ARVC PMID 31345875 IIa + HRS 2017 PMID 28219760 — β-blocker first-line for arrhythmia suppression in ARVC; nadolol or metoprolol succinate equivalent options
2. metoprolol succinate ARVC long-term alternate: metoprolol succinate 25-200 mg PO daily titrated PO daily; lifelong (selective_beta1_blocker_extended_release, first line) — HRS 2019 ARVC IIa; preferred in LV-dominant / biventricular phenotype with HF (β1-selective, GDMT pillar)
3. sotalol ARVC sustained VT or post-ICD-shock: sotalol 80-160 mg PO BID (renal dose-adjustment if eGFR <60; AVOID if eGFR <40) PO BID (class_iii_antiarrhythmic_with_beta_blocker, add on) — HRS 2017 IIa + HRS 2019 ARVC IIa — Class III + β-blocker dual mechanism; QTc surveillance mandatory; K ≥4.5 + Mg ≥2.0 mandatory; renal dose-adjustment critical
4. magnesium sulfate 2 g IV over 5–15 min then 2 g/h infusion supportive for polymorphic VT + shivering suppression during TTM rewarm IV continuous (electrolyte_membrane_stabilizer, first line) — AHA 2020 ACLS supportive for polymorphic VT; mandatory target ≥2.0 with sotalol initiation (QT prolongation safety)
5. potassium chloride 20–40 mEq IV/PO until K ≥4.0 standard (≥4.5 mandatory if on sotalol) IV/PO PRN until target sustained (electrolyte, first line) — Standard polymorphic VT supportive therapy; mandatory ≥4.5 with sotalol (Class III QT-prolonging)
6. norepinephrine 0.05–0.5 µg/kg/min titrate MAP ≥65; cautious in ARVC (sympathomimetic load on weakened desmosomes); minimize dose; substitute vasopressin or phenylephrine when feasible IV continuous (vasopressor_alpha1_beta1, first line) — SOAP-II PMID 20200382; first-line post-ROSC vasoactive; in ARVC use lowest effective dose to minimize sympathomimetic stress on desmosomal junctions
7. vasopressin 0.03 U/min IV fixed dose (catecholamine-sparing alternative) IV continuous (vasopressor_v1_agonist, second line) — Non-adrenergic vasopressor preferred adjunct in ARVC to minimize sympathomimetic stress on weakened desmosomes
8. milrinone 0.125–0.5 µg/kg/min IV (catecholamine-sparing inotrope alternative for biventricular ARVC with HF) IV continuous (phosphodiesterase_3_inhibitor_inodilator, comorbidity specific) — PDE3 pathway — non-catecholamine inotrope; preferred substitute for dobutamine in ARVC to minimize sympathomimetic stress; particularly useful in biventricular phenotype with HF
9. epinephrine 1 mg IV q3–5 min during arrest only (ACLS standard); minimize post-ROSC infusion if alternative IV standard ACLS only (inotrope_chronotrope_vasopressor, first line) — AHA 2020 ACLS — standard arrest pathway; minimize post-ROSC infusion to limit sympathomimetic load
10. propofol 5–50 µg/kg/min; titrate RASS IV continuous (sedative_iv_anesthetic, first line) — PADIS 2018; preferred sedative for post-ROSC TTM (no specific ARVC contraindication unlike Brugada)
11. fentanyl 25–200 µg/h IV continuous (opioid_analgesic, first line) — PADIS 2018; preferred opioid for analgesia + shivering suppression during TTM rewarm
12. dexmedetomidine 0.2–1.4 µg/kg/h; no bolus IV continuous (alpha2_agonist_sedative, second line) — PADIS 2018; α2 agonist actually REDUCES central sympathetic outflow — useful in ARVC for shivering suppression + delirium prevention
13. acetaminophen 650 mg PO/PR/IV q6h PRN PO/PR/IV q6h PRN (antipyretic_analgesic, first line) — Standard analgesia / antipyresis; AVOID NSAIDs (fluid retention + RV adverse remodeling concern in ARVC HF)
14. lidocaine 1–1.5 mg/kg IV bolus then 1–4 mg/min infusion bridge for refractory polymorphic VT during arrest pathway IV continuous bridge (class_ib_sodium_channel_blocker, rescue) — AHA 2020 ACLS standard polymorphic VT bridge; acceptable in ARVC (unlike Brugada where contraindicated); β-blocker + sotalol preferred long-term
15. amiodarone 150-300 mg IV bolus then 1 mg/min × 6 h then 0.5 mg/min × 18 h for refractory VT bridge IV continuous bridge (class_iii_antiarrhythmic, rescue) — AHA 2020 ACLS for VT/VF; acceptable bridge in ARVC; long-term sotalol preferred per HRS 2019 IIa

Setting playbook (outpatient) — Long-term EP / inherited-arrhythmia clinic surveillance; ICD interrogation q3–6 mo; β-blocker maintenance; sotalol if on therapy + QTc surveillance + level monitoring; catheter ablation evaluation for refractory storm or recurrent ICD shocks; family cascade testing completion + ongoing identification of newly recognised relatives (cardiac MRI for relatives is critical element); LIFELONG endurance-exercise + competitive-sports avoidance + identity-loss support; advanced HF therapies referral including transplant for end-stage biventricular / LV-dominant phenotype (DSP / PLN); annual mental health screen
16. continue β-blocker maintenance lifelong nadolol 1-1.5 mg/kg/d OR metoprolol succinate 25-200 mg/d PO daily; lifelong — ARVC diagnosis (HRS 2019 ARVC IIa)
17. continue sotalol lifelong if on therapy with QTc surveillance 80-160 mg PO BID (renal dose-adjustment) PO BID; lifelong — sustained VT or post-ICD-shock burden (HRS 2019 + HRS 2017 IIa)
18. continue lifelong avoidance of class I antiarrhythmics + endurance exercise + competitive sports patient education + curated counseling n/a lifelong — ARVC diagnosis (CAST + HRS 2019 + ITF 2020 Class I)
19. GDMT 4-pillar for biventricular / LV-dominant ARVC with HFrEF (ARNI + β-blocker + MRA + SGLT2i) per HF guideline-directed PO daily; lifelong — biventricular / LV-dominant ARVC with HFrEF (HRS 2019 ARVC IIa + HF guideline directed therapy)

Non-pharmacologic actions:
- EP / inherited-arrhythmia clinic q3–6 mo lifelong
- ICD generator / lead surveillance per device clinic; replacement at end-of-service
- Catheter ablation evaluation at expert ARVC center for refractory storm or recurrent ICD shocks despite β-blocker + sotalol (combined endo + epicardial substrate ablation)
- Family cascade testing — ongoing identification of newly recognised relatives (extended pedigree); cardiac MRI for relatives is critical element
- Lifestyle: LIFELONG AVOIDANCE OF ENDURANCE EXERCISE + COMPETITIVE SPORTS (HRS 2019 + ITF 2020 Class I); recreational low-intensity activity ≤6 METs titrated to symptoms; identity-loss support for endurance athletes
- Medic-alert bracelet maintenance — "ARVC — endurance exercise contraindicated; ICD in place"
- Advanced HF therapies referral including transplant evaluation for end-stage biventricular / LV-dominant phenotype (DSP / PLN variants)
- Mental health long-term — high risk identity-loss in endurance athletes

AVOID / contraindication checks:
- Class_i_antiarrhythmics_AVOID_in_arvc (flecainide, propafenone — proarrhythmic in scar substrate per CAST framework; structural heart disease contraindication)
- High_dose_norepinephrine_minimize_in_arvc (sympathomimetic stress on weakened desmosomes; substitute vasopressin or low dose phenylephrine when feasible)
- Dobutamine_substitute_milrinone_in_arvc (catecholamine inotrope — substitute non catecholamine PDE3 inhibitor milrinone for inotropic support especially in biventricular phenotype with HF)
- Nsaids_AVOID_in_arvc_with_hf (fluid retention + renal effects + adverse RV remodeling concern)
- Digoxin_avoid_in_arvc (narrow therapeutic window in cardiomyopathy; AV nodal blockade not the primary management strategy)
- Endurance_exercise_AVOID_lifelong_in_arvc (HRS 2019 + ITF 2020 Class I — THE most disease modifying intervention; James 2013 + Saberniak 2014 + Sawant 2014 — endurance exercise accelerates fibrofatty replacement)
- Competitive_sports_AVOID_lifelong_in_arvc (HRS 2019 + ITF 2020 Class I)
- Beta_blocker_LIFELONG_FIRST_LINE_in_arvc (nadolol or metoprolol succinate; HRS 2019 IIa)
- Sotalol_FOR_SUSTAINED_VT_OR_POST_ICD_SHOCK_in_arvc (HRS 2019 + HRS 2017 IIa — Class III + β blocker dual mechanism; renal dose adjustment critical; K ≥4.5 + Mg ≥2.0 mandatory)
- Icd_required_HRS_2019_arvc_class_i_post_arrest (sustained VT/VF survivor — secondary prevention; subcutaneous ICD considered if no pacing indication)
- Catheter_ablation_at_expert_arvc_center_for_refractory_storm (combined endo + epicardial — endocardial alone insufficient because scar is epicardial / midwall in ARVC)
- Cardiac_mri_at_4_to_6_wk_GOLD_STANDARD_DIAGNOSTIC (allows post arrest stunning to resolve; LGE pattern critical for diagnosis + LV dominant variant identification)
- Lv_dominant_biventricular_phenotype_HF_management_overlay (DSP, PLN variants — GDMT 4 pillar, advanced HF therapies including transplant for end stage)

Monitoring

Regimen monitoring:
- continuous ecg telemetry x 48-72h (HRS 2019 ARVC)
- serial ecg q4-6h x 24h then q6-8h x 48h (track T-wave inversion + epsilon waves + QTc trend if on sotalol)
- BMP q4-6h until K above 4 and Mg above 2 sustained (mandatory targets — ≥4.5 if on sotalol)
- serial troponin q3-6h x 24h (4th UDMI 2018)
- continuous core temp via bladder or esophageal probe during TTM (TTM2 PMID 34133859)
- aggressive shivering control during ttm rewarm (Mg + acetaminophen + dexmedetomidine — minimize sympathomimetic stress)
- continuous EEG for 24-48h (Sandroni 2021 PMID 33745427)
- NSE at 24h 48h 72h (Sandroni 2021)
- lactate q2-4h until normalized (SCAI 2022 PMID 35718438)
- CARDIAC MRI at 4 to 6 wk GOLD STANDARD DIAGNOSTIC LGE pattern
- genetic panel pkp2 dsp dsg2 jup dsc2 tmem43 pln core then expanded if negative (HRS 2019 ARVC PMID 31345875)
- first degree relative ECG AND ECHO AND CARDIAC MRI AND HOLTER AND EXERCISE STRESS TEST screening referral (cascade testing — multimodality)
- icd interrogation q3 to 6 mo lifelong with appropriate therapy log review
- sotalol QT q4-6h with K 4.5 target if on therapy

Setting (outpatient) monitoring:
- q3–6 mo ICD interrogation lifelong
- Annual ECG
- Annual Holter if β-blocker / sotalol titration or symptoms
- Annual cardiac MRI for disease progression surveillance
- Annual QTc + K + Mg if on sotalol
- Family cascade testing progress documentation
- Lifelong drug + endurance-exercise audit at every clinic visit

Follow-up plan: Cardiology + EP / inherited-arrhythmia clinic at 2–4 wks; CARDIAC MRI at 4–6 wk (gold-standard diagnostic; allows post-arrest stunning to resolve); GENETIC PANEL completed (PKP2/DSP/DSG2/JUP/DSC2/TMEM43/PLN); 2020 PADUA CRITERIA application + classification (definite ARVC, borderline, possible); CASCADE FAMILY SCREENING — first-degree relatives ECG + echo + cardiac MRI + signal-averaged ECG + 24-h Holter + exercise stress test + genotyping at proband mutation; β-blocker maintenance + sotalol if indicated; ICD interrogation q3–6 mo; CATHETER ABLATION at expert ARVC ablation center for refractory storm or recurrent ICD shocks despite sotalol (combined endo + epicardial substrate ablation); LIFELONG AVOIDANCE OF ENDURANCE EXERCISE + COMPETITIVE SPORTS (HRS 2019 + ITF 2020 Class I — THE most disease-modifying intervention; James 2013 + Saberniak 2014 + Sawant 2014); recreational low-intensity activity ≤6 METs titrated to symptoms; LV-dominant / biventricular phenotype HF management overlay (GDMT 4-pillar) with advanced HF therapies including transplant for end-stage; medic-alert bracelet "ARVC — endurance exercise contraindicated"; PTSD / mental health screen
- Close-out criterion: cardiology + EP follow-up + cardiac MRI + Padua criteria classification + genetic panel + cascade family screening + ICD + β-blocker + sotalol + endurance-exercise avoidance + LV-dominant HF management + mental health all booked / documented

Monitoring phase: Continuous telemetry × 48–72 h; A-line; multimodal neuroprog ≥72 h post-rewarm (Sandroni 2021 PMID 33745427); serial ECG q4–6 h × 24 h then q6–8 h × 48 h; if on sotalol QT q4-6 h with K ≥4.5 mandatory; daily medication audit; β-blocker initiation logging; cascade-screening referral status documented

Disposition

Current setting: outpatient — Long-term EP / inherited-arrhythmia clinic surveillance; ICD interrogation q3–6 mo; β-blocker maintenance; sotalol if on therapy + QTc surveillance + level monitoring; catheter ablation evaluation for refractory storm or recurrent ICD shocks; family cascade testing completion + ongoing identification of newly recognised relatives (cardiac MRI for relatives is critical element); LIFELONG endurance-exercise + competitive-sports avoidance + identity-loss support; advanced HF therapies referral including transplant for end-stage biventricular / LV-dominant phenotype (DSP / PLN); annual mental health screen

Disposition criteria:
- Long-term continuation lifelong; ICD + β-blocker + sotalol if indicated + lifelong endurance-exercise avoidance + family cascade testing operational + GDMT 4-pillar for biventricular / LV-dominant ARVC with HFrEF + advanced HF / transplant evaluation as needed

Escalation triggers (move to higher acuity):
- Recurrent ICD shock → emergent EP + storm investigation; catheter ablation evaluation expedited at expert ARVC center
- Significant LV dysfunction progression on cardiac MRI → advanced HF clinic; transplant evaluation if end-stage
- β-blocker / sotalol intolerance → catheter ablation evaluation; alternate β-blocker
- Family member positive screening on cardiac MRI → cascade testing extended + EP referral
- Mental health crisis → psychiatry; identity-loss support
- Endurance-exercise non-compliance → reinforce education + family meeting + EP counseling; long-term progression risk emphasized

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Recurrent VT (typically LBBB superior axis morphology = RV-origin) post-ROSC suggests ongoing ARVC storm physiology — esmolol IV bridge → metoprolol/nadolol PO + magnesium + sotalol bridge once stable; AVOID class I antiarrhythmics (flecainide, propafenone — proarrhythmic in scar substrate); refractory storm → urgent transfer to expert ARVC ablation center for combined endo + epicardial substrate ablation
- [SEVERE] Inadvertent administration of class I antiarrhythmic (flecainide, propafenone) post-ROSC in confirmed or suspected ARVC — STOP drug + escalate to EP + chart audit + nursing handoff review; substitute amiodarone or lidocaine bridge if needed; class I antiarrhythmics are proarrhythmic in scar substrate per CAST framework
- [SEVERE] Sustained VT/VF survivor by definition meets HRS 2019 ARVC + ITF 2020 Class I ICD criteria as SECONDARY PREVENTION — implant pre-discharge or schedule within 1 wk; subcutaneous ICD considered if no pacing indication (RV-lead-related issues common with desmosomal disease); WCD bridge if ICD deferred for stabilization

Citations

- 2020 Padua Criteria (Corrado PMID 31676124) + HRS 2019 ARVC genetic / management statement (Towbin PMID 31345875) + ITF 2020 ICD therapy in ARVC (Cadrin-Tourigny PMID 30852048) + HRS 2017 Inherited Arrhythmia Syndromes (Al-Khatib PMID 28219760) + AHA 2020 ACLS / Post-Cardiac-Arrest Care + TTM2 + Sandroni 2021 ERC-ESICM neuroprog + James 2013 / Saberniak 2014 / Sawant 2014 endurance exercise as disease modifier + ESC 2022 VA / SCD prevention [PMID:31676124](https://pubmed.ncbi.nlm.nih.gov/31676124/)
- Cited evidence (PMID 31345875) [PMID:31345875](https://pubmed.ncbi.nlm.nih.gov/31345875/)
- Cited evidence (PMID 30852048) [PMID:30852048](https://pubmed.ncbi.nlm.nih.gov/30852048/)
- Cited evidence (PMID 28219760) [PMID:28219760](https://pubmed.ncbi.nlm.nih.gov/28219760/)
- Cited evidence (PMID 23394677) [PMID:23394677](https://pubmed.ncbi.nlm.nih.gov/23394677/)

Last reconciled with current guidelines: 2026-05-15.
References
  • 2020 Padua Criteria (Corrado PMID 31676124) + HRS 2019 ARVC genetic / management statement (Towbin PMID 31345875) + ITF 2020 ICD therapy in ARVC (Cadrin-Tourigny PMID 30852048) + HRS 2017 Inherited Arrhythmia Syndromes (Al-Khatib PMID 28219760) + AHA 2020 ACLS / Post-Cardiac-Arrest Care + TTM2 + Sandroni 2021 ERC-ESICM neuroprog + James 2013 / Saberniak 2014 / Sawant 2014 endurance exercise as disease modifier + ESC 2022 VA / SCD prevention — PMID:31676124
  • Cited evidence (PMID 31345875) — PMID:31345875
  • Cited evidence (PMID 30852048) — PMID:30852048
  • Cited evidence (PMID 28219760) — PMID:28219760
  • Cited evidence (PMID 23394677) — PMID:23394677