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cardio.post-arrest.cpvt-channelopathy.v1PRODUCTION
cardio.post-arrest.cpvt-channelopathy.v1

Post-cardiac-arrest care — CPVT channelopathy (RYR2 / CASQ2; exercise- or emotion-triggered bidirectional VT → VF arrest)

cardiologyacuteadultpediatric
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12/12 authored

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

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Detailed

Recognize post-ROSC + exercise- or emotion-triggered arrest in young patient + normal resting ECG + structurally normal heart as CPVT-arrest cohort; pivot from generic post-arrest care to channelopathy-specific avoidance protocol; route to parent cardio.post-arrest.core.v1 for TTM + neuroprog while specializing on exercise-stress ECG provocation + family screening + nadolol + flecainide adjunct + LCSD + ICD + competitive-sports avoidance arcs

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CPVT high pretest probability confirmed + structural disease screen initiated

Patient inputs (25)

CPVT typically manifests in childhood / adolescence / young adulthood (median age first event ~10–14 y; ~80% by age 40); pediatric resuscitation modifications below age 8; ICD device-size considerations in pediatric / small-frame patients; nadolol weight-based dosing 1.5–2.0 mg/kg/d

Equal sex distribution unlike Brugada (8:1 male) or LQT2 (female event predominance); informs cascade-screening prioritization

Trigger pattern is highly informative and pathognomonic — exertion (running, swimming, competitive sports) or intense emotion (fright, surprise, anger) is the CPVT signature; informs lifelong sports-avoidance + emotion-trigger management; drives genetic panel + exercise-stress ECG referral

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

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

VF most common terminal rhythm (~85–90%); bidirectional or polymorphic VT degenerating to VF is the classic CPVT mechanism; informs storm-suppression bridge planning

Family history of SCD <40 y, known CPVT (autosomal dominant RYR2 in ~55-65%; recessive CASQ2 in ~3-5%), or familial pattern of exercise-triggered syncope is critical input — drives cascade exercise-stress ECG screening of relatives + genetic referral

Prior exercise- or emotion-triggered syncope is a classic missed-opportunity diagnosis for CPVT (often misattributed to vasovagal); strong pretest probability marker

Comprehensive medication review for sympathomimetics (decongestants pseudoephedrine), amphetamines (ADHD stimulants methylphenidate / dextroamphetamine), illicit substances (cocaine, MDMA), high-dose caffeine — STOP all offenders + document; informs whether surge unmasked CPVT substrate

eGFR for nadolol dose adjustment (renally cleared — half-life prolonged in CKD; reduce dose if eGFR <50)

EXERCISE STRESS ECG (treadmill or bicycle) > resting ECG > epinephrine challenge for CPVT diagnosis; reproduces bidirectional or polymorphic VT in ~75% of true CPVT cases even with normal resting ECG; performed in inherited-arrhythmia center with continuous ECG + defibrillator pads + EP supervision; cascade screening of relatives also uses exercise stress test

Targeted gene panel — RYR2 core (~55–65% positive), CASQ2 (~3–5% recessive), expanded panel CALM1/2/3, KCNJ2, TRDN, TECRL if family history positive or phenotype atypical; informs prognosis + cascade testing of relatives at proband mutation; per HRS 2017 PMID 28219760

Resting ECG is typically NORMAL in CPVT (no QT prolongation, no V1-V3 coved ST, no epsilon waves) — this normalcy is itself diagnostic when paired with exercise-trigger phenotype; serial ECGs document QTc + baseline morphology before exercise stress provocation

Rule out structural disease (ARVC most important differential — RV-dominant cardiomyopathy can present with exertion-triggered VT; HCM, anomalous coronary, infiltrative); CPVT heart is structurally normal — structural finding triggers alternate or overlap pathway

Often modestly elevated from arrest + CPR; rise pattern helps differentiate from ACS-mediated arrest; primary CPVT 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 supportive; hypokalemia not the dominant CPVT trigger (vs LQT) but still avoided

Mg target ≥2.0 supportive; replace standard polymorphic VT supportive therapy + shivering suppression during rewarm

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

MAP ≥65 target post-ROSC; SCAI staging if shock; CAUTIOUS vasopressor in CPVT — minimize epinephrine + NE because catecholamine surge is THE trigger; vasopressin or low-dose phenylephrine preferred substitute when feasible

Bradycardia is NOT a CPVT trigger (unlike LQT3) — DO NOT use isoproterenol (catecholamine — directly triggers RyR2 leak); pacing for symptomatic bradycardia per standard ACLS

TTM target 33–37.5 °C × 24 h (TTM2 PMID 34133859); shivering during rewarm is a catecholamine surge trigger — aggressive shivering control with magnesium + acetaminophen + buspirone + adequate sedation

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

Sympathomimetic / catecholaminergic substances are direct CPVT triggers; tox screen at presentation informs acquired vs unmasked CPVT phenotype + lifestyle counseling

Cardiac MRI at 4–6 wk post-arrest if echo equivocal — RULE OUT ARVC overlap (key differential — exertion-triggered VT in young patient overlaps phenotypically), HCM, infiltrative; allows post-arrest stunning to resolve before assessment

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

Severity triggers (6)

6 need judgement
  • informationallife_threateningrecurrent_bidirectional_polymorphic_vt_post_rosc_storm_bridge
    Recurrent bidirectional / polymorphic VT or VF post-ROSC suggests ongoing CPVT storm physiology — esmolol IV bridge + magnesium + flecainide PO load + urgent EP for LCSD evaluation; AVOID isoproterenol (CONTRAINDICATED — directly triggers RyR2 leak); minimize epinephrine + NE
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinadvertent_catecholamine_administered_post_rosc
    Inadvertent administration of catecholamine drug post-ROSC (isoproterenol, dobutamine, high-dose epinephrine infusion, sympathomimetic decongestant, amphetamine) in confirmed or suspected CPVT — STOP drug + escalate to EP + chart audit + nursing handoff review; substitute with catecholamine-sparing alternative (vasopressin, phenylephrine, milrinone)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereshivering_during_ttm_rewarm_cpvt_trigger
    Shivering during TTM rewarm in CPVT post-arrest is a catecholamine surge trigger — aggressive shivering control with magnesium + acetaminophen + buspirone + adequate sedation; if refractory, propofol bolus rescue + neuromuscular blockade (last-line); slow rewarm 0.25–0.5 °C/h
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereicd_eligibility_evaluation_cpvt_post_arrest_with_programming_caution
    Sustained VT/VF survivor by definition meets HRS 2017 Class I ICD criteria — implant pre-discharge or schedule within 1 wk; CRITICAL — program ICD with long detection windows + ATP-first + high therapy thresholds because ICD shocks themselves trigger catecholamine surge that can PROVOKE further VT/VF (so-called "shock-storm"); ICD WITHOUT optimal nadolol + flecainide + LCSD is INADEQUATE alone in CPVT; subcutaneous ICD considered if no pacing indication; WCD bridge if ICD deferred for stabilization
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecascade_family_screening_with_exercise_stress_test_referral_required
    Confirmed CPVT (or strongly suspected pending genetic results) triggers mandatory cascade screening of first-degree relatives — ECG + EXERCISE STRESS TEST (highest yield — far superior to resting ECG which is normal in CPVT) + genetic testing at proband mutation; genetic counseling referral; many newly identified relatives are asymptomatic carriers requiring lifelong management
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverelcsd_evaluation_for_refractory_cpvt
    Refractory CPVT despite optimal nadolol + flecainide adjunct, β-blocker intolerance, or significant ICD-shock burden → LCSD (left cardiac sympathetic denervation) evaluation per HRS 2017 Class IIa — the most effective non-pharmacologic intervention for refractory CPVT; performed at LCSD-capable inherited-arrhythmia center
    Trigger could not be auto-evaluated — needs clinician judgement.

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TREATMENTrequiredDrives monitoring threshold
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Recommended regimen

CPVT post-arrest phenotype — standard post-ROSC bundle with cautious catecholaminergic minimization + aggressive shivering control + LONG-TERM nadolol (preferred β-blocker per CPVT registry) + flecainide adjunct + ICD pathway (HRS 2017 Class I) + LCSD for refractory + cascade family exercise-stress-ECG screening
axis: cpvt_post_arrest_phenotype
Selected axis "CPVT post-arrest phenotype — standard post-ROSC bundle with cautious catecholaminergic minimization + aggressive shivering control + LONG-TERM nadolol (preferred β-blocker per CPVT registry) + flecainide adjunct + ICD pathway (HRS 2017 Class I) + LCSD for refractory + cascade family exercise-stress-ECG screening" by default fallback (first axis)
  • nadolol
    first line
    non_selective_beta_blocker_long_acting
    CPVT long-term: nadolol 1.5–2.0 mg/kg/d PO daily (preferred over propranolol or metoprolol per Roston / Mazzanti CPVT registry meta-analysis — superior arrhythmia suppression; the only β-blocker with consistent registry-level efficacy) • PO • daily; lifelong
    triggers: confirmed_or_high_pretest_cpvt_post_arrest, storm_controlled_initiate_long_term_bb
    HRS 2017 PMID 28219760 Class I + Roston CPVT registry PMID 27406239 + Mazzanti 2018 JACC PMID 29804673 — nadolol provides superior arrhythmia suppression vs propranolol or metoprolol; first-line lifelong therapy in CPVT
    rxcui 7226
  • propranolol
    second line
    non_selective_beta_blocker
    CPVT alternate (nadolol unavailable): propranolol 2–4 mg/kg/d divided BID-QID • PO • BID-QID; lifelong
    triggers: nadolol_unavailable, cpvt_long_term
    HRS 2017 Class I; Mazzanti 2018 — historical first-line; less effective than nadolol per Roston meta-analysis; QID dosing reduces adherence
    rxcui 8787
  • flecainide
    add on
    class_ic_sodium_channel_blocker_with_ryr2_blockade
    CPVT adjunct to nadolol: flecainide 100–300 mg/d PO divided BID (target trough 0.2–1.0 µg/mL) • PO • BID; long-term
    triggers: cpvt_with_recurrent_events_on_optimal_nadolol, high_risk_cpvt_genotype_phenotype
    van der Werf 2011 JACC PMID 21962432 + Watanabe 2009 Nat Med PMID 19620991 — flecainide directly blocks RyR2 in addition to Na channel, reducing diastolic Ca²⁺ leak; HRS 2017 IIa adjunct on top of β-blocker; reduces arrhythmia events
    rxcui 4441
  • 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
    AHA 2020 ACLS supportive for polymorphic VT; shivering control during TTM rewarm is critical in CPVT (shivering = catecholamine surge trigger); Mg first-line for shivering suppression
    rxcui 6585
  • potassium chloride
    first line
    electrolyte
    20–40 mEq IV/PO until K ≥4.0 supportive (not the dominant CPVT trigger but standard support) • IV/PO • PRN until target sustained
    triggers: k_below_4.0_post_rosc
    Standard polymorphic VT supportive therapy
    rxcui 8591
  • norepinephrine
    first line
    vasopressor_alpha1_beta1
    0.05–0.5 µg/kg/min titrate MAP ≥65; CAUTION in CPVT (catecholamine load); minimize dose; substitute vasopressin or phenylephrine when feasible • IV • continuous
    triggers: post_rosc_vasoplegia_when_no_alternative
    SOAP-II PMID 20200382; first-line post-ROSC vasoactive when needed; in CPVT cohort use lowest effective dose because adrenergic load triggers RyR2 leak; consider vasopressin / phenylephrine substitute when feasible
    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_cpvt_minimize_catecholamines
    VANISH-style catecholamine-sparing alternative; non-adrenergic vasopressor preferred in CPVT to minimize RyR2 trigger load
    rxcui 11149
  • phenylephrine
    second line
    vasopressor_alpha1_pure
    0.5–10 µg/kg/min IV titrate MAP • IV • continuous
    triggers: post_rosc_vasoplegia_in_cpvt_minimize_beta_load
    Pure α1 vasopressor without β-1 stimulation — preferred substitute in CPVT to minimize adrenergic trigger; useful when NE not tolerated
    rxcui 8163
  • epinephrine
    first line
    inotrope_chronotrope_vasopressor
    1 mg IV q3–5 min during arrest only (ACLS standard); AVOID post-ROSC infusion if alternative; CONTRAINDICATED chronic in CPVT • IV • standard ACLS only
    triggers: cardiac_arrest_during_index_event
    AHA 2020 ACLS — standard arrest pathway; in confirmed CPVT post-ROSC AVOID subsequent infusions because epinephrine is the prototypical CPVT trigger
    rxcui 3992
  • milrinone
    comorbidity specific
    phosphodiesterase_3_inhibitor_inodilator
    0.125–0.5 µg/kg/min IV (catecholamine-sparing inotrope alternative) • IV • continuous
    triggers: post_rosc_inotropic_support_avoiding_dobutamine_in_cpvt
    PDE3 pathway — non-catecholamine inotrope; preferred substitute for dobutamine in CPVT to minimize adrenergic trigger load; vasodilator + inotrope
    rxcui 52769
  • midazolam
    first line
    benzodiazepine_sedative
    0.02–0.1 mg/kg/h IV; titrate RASS • IV • continuous
    triggers: post_rosc_intubation_ttm, shivering_control_adjunct
    PADIS 2018; benzodiazepine for sedation + shivering suppression during TTM rewarm — adequate sedation depth blunts catecholamine surge
    rxcui 6960
  • 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 sedation adjunct in CPVT for shivering suppression + delirium prevention
    rxcui 48937
  • acetaminophen
    first line
    antipyretic_analgesic
    650 mg PO/PR/IV q6h scheduled (also shivering suppression during TTM rewarm) • PO/PR/IV • q6h scheduled
    triggers: shivering_during_ttm_rewarm, fever_post_arrest, analgesia_avoid_nsaids_in_aki
    Standard analgesia / antipyresis + shivering suppression component; QT-neutral and catecholamine-neutral
    rxcui 161
  • buspirone
    add on
    serotonin_5ht1a_partial_agonist
    30 mg PO BID for shivering suppression (off-label TTM adjunct per BAIR-Hugger / shivering-control protocols) • PO • BID
    triggers: shivering_during_ttm_rewarm_in_cpvt
    Serotonergic shivering suppression off-label TTM adjunct; non-catecholaminergic; Choi-style anti-shivering ladder
    rxcui 1827
  • 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 only • IV • continuous bridge
    triggers: refractory_polymorphic_vt_during_arrest_no_alternative
    AHA 2020 ACLS standard polymorphic VT bridge; acceptable in CPVT (unlike Brugada where contraindicated); β-blocker + flecainide preferred long-term
    rxcui 6387

outpatient playbook — drug actions (3)

  1. 1. continue nadolol maintenance lifelong (preferred CPVT β-blocker)
    rxcui 7226
    nadolol 1.5–2.0 mg/kg/d (weight-based titration in growing pediatric) • PO • daily; lifelong
    trigger: CPVT diagnosis
    HRS 2017 Class I + Roston/Mazzanti registry
  2. 2. continue flecainide adjunct lifelong if on therapy
    rxcui 4441
    100–300 mg/d PO BID (target trough 0.2–1.0 µg/mL) • PO • BID; lifelong
    trigger: high-risk CPVT or recurrent events on nadolol alone
    HRS 2017 IIa + van der Werf JACC 2011
  3. 3. continue lifelong avoidance of sympathomimetics + amphetamines + cocaine + MDMA + high-dose caffeine
    patient education + curated counseling • n/a • lifelong
    trigger: CPVT diagnosis
    HRS 2017

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: ROSC after out-of-hospital VF arrest with known CPVT (prior diagnosis, prior exercise-triggered syncope, family history) or with sentinel pre-arrest narrative — exertion- or emotion-triggered collapse in a young patient with normal resting ECG and structurally normal heart on echo; Witnessed arrest during exercise (running, swimming, sports) or intense emotion (fright, anger, surprise) in a previously healthy young patient (childhood–young adulthood) with normal resting 12-lead ECG and normal echo — high CPVT pretest probability; classic phenotype; Family history of sudden death <40 y, known CPVT in first-degree relative, or familial pattern of exercise-triggered syncope — autosomal dominant RYR2 most common (~55-65%); offer genetic panel + cascade screening with EXERCISE STRESS TEST (highest yield).

Objective

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

Assessment

**Post-cardiac-arrest care — CPVT channelopathy (RYR2 / CASQ2; exercise- or emotion-triggered bidirectional VT → VF arrest)** (cardio.post-arrest.cpvt-channelopathy.v1).
Phenotype framing: CPVT (RYR2 / CASQ2 — exercise- or emotion-triggered bidirectional VT in young patient with normal resting ECG and structurally normal heart) vs ARVC (RV-dominant CMP with epsilon waves and structural changes on cardiac MRI — key differential for exertion-triggered VT in young) vs LQT (prolonged QTc on resting ECG — different channelopathy mechanism) vs Brugada (V1-V3 coved ST + sleep / fever trigger — different channelopathy) vs digoxin toxicity (bidirectional VT with elevated digoxin level + clinical context — non-channelopathic mimic) vs Andersen-Tawil syndrome (KCNJ2 — periodic paralysis + dysmorphic features + bidirectional VT overlap) vs idiopathic VF — this differential drives long-term plan + family screening
Scope: Recognize post-ROSC + exercise- or emotion-triggered arrest in young patient + normal resting ECG + structurally normal heart as CPVT-arrest cohort; pivot from generic post-arrest care to channelopathy-specific avoidance protocol; route to parent cardio.post-arrest.core.v1 for TTM + neuroprog while specializing on exercise-stress ECG provocation + family screening + nadolol + flecainide adjunct + LCSD + ICD + competitive-sports avoidance arcs

No severity triggers fired against current inputs.

Plan

Regimen axis: **CPVT post-arrest phenotype — standard post-ROSC bundle with cautious catecholaminergic minimization + aggressive shivering control + LONG-TERM nadolol (preferred β-blocker per CPVT registry) + flecainide adjunct + ICD pathway (HRS 2017 Class I) + LCSD for refractory + cascade family exercise-stress-ECG screening**.
1. nadolol CPVT long-term: nadolol 1.5–2.0 mg/kg/d PO daily (preferred over propranolol or metoprolol per Roston / Mazzanti CPVT registry meta-analysis — superior arrhythmia suppression; the only β-blocker with consistent registry-level efficacy) PO daily; lifelong (non_selective_beta_blocker_long_acting, first line) — HRS 2017 PMID 28219760 Class I + Roston CPVT registry PMID 27406239 + Mazzanti 2018 JACC PMID 29804673 — nadolol provides superior arrhythmia suppression vs propranolol or metoprolol; first-line lifelong therapy in CPVT
2. propranolol CPVT alternate (nadolol unavailable): propranolol 2–4 mg/kg/d divided BID-QID PO BID-QID; lifelong (non_selective_beta_blocker, second line) — HRS 2017 Class I; Mazzanti 2018 — historical first-line; less effective than nadolol per Roston meta-analysis; QID dosing reduces adherence
3. flecainide CPVT adjunct to nadolol: flecainide 100–300 mg/d PO divided BID (target trough 0.2–1.0 µg/mL) PO BID; long-term (class_ic_sodium_channel_blocker_with_ryr2_blockade, add on) — van der Werf 2011 JACC PMID 21962432 + Watanabe 2009 Nat Med PMID 19620991 — flecainide directly blocks RyR2 in addition to Na channel, reducing diastolic Ca²⁺ leak; HRS 2017 IIa adjunct on top of β-blocker; reduces arrhythmia events
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; shivering control during TTM rewarm is critical in CPVT (shivering = catecholamine surge trigger); Mg first-line for shivering suppression
5. potassium chloride 20–40 mEq IV/PO until K ≥4.0 supportive (not the dominant CPVT trigger but standard support) IV/PO PRN until target sustained (electrolyte, first line) — Standard polymorphic VT supportive therapy
6. norepinephrine 0.05–0.5 µg/kg/min titrate MAP ≥65; CAUTION in CPVT (catecholamine load); minimize dose; substitute vasopressin or phenylephrine when feasible IV continuous (vasopressor_alpha1_beta1, first line) — SOAP-II PMID 20200382; first-line post-ROSC vasoactive when needed; in CPVT cohort use lowest effective dose because adrenergic load triggers RyR2 leak; consider vasopressin / phenylephrine substitute when feasible
7. vasopressin 0.03 U/min IV fixed dose (catecholamine-sparing alternative) IV continuous (vasopressor_v1_agonist, second line) — VANISH-style catecholamine-sparing alternative; non-adrenergic vasopressor preferred in CPVT to minimize RyR2 trigger load
8. phenylephrine 0.5–10 µg/kg/min IV titrate MAP IV continuous (vasopressor_alpha1_pure, second line) — Pure α1 vasopressor without β-1 stimulation — preferred substitute in CPVT to minimize adrenergic trigger; useful when NE not tolerated
9. epinephrine 1 mg IV q3–5 min during arrest only (ACLS standard); AVOID post-ROSC infusion if alternative; CONTRAINDICATED chronic in CPVT IV standard ACLS only (inotrope_chronotrope_vasopressor, first line) — AHA 2020 ACLS — standard arrest pathway; in confirmed CPVT post-ROSC AVOID subsequent infusions because epinephrine is the prototypical CPVT trigger
10. milrinone 0.125–0.5 µg/kg/min IV (catecholamine-sparing inotrope alternative) IV continuous (phosphodiesterase_3_inhibitor_inodilator, comorbidity specific) — PDE3 pathway — non-catecholamine inotrope; preferred substitute for dobutamine in CPVT to minimize adrenergic trigger load; vasodilator + inotrope
11. midazolam 0.02–0.1 mg/kg/h IV; titrate RASS IV continuous (benzodiazepine_sedative, first line) — PADIS 2018; benzodiazepine for sedation + shivering suppression during TTM rewarm — adequate sedation depth blunts catecholamine surge
12. fentanyl 25–200 µg/h IV continuous (opioid_analgesic, first line) — PADIS 2018; preferred opioid for analgesia + shivering suppression during TTM rewarm
13. 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 sedation adjunct in CPVT for shivering suppression + delirium prevention
14. acetaminophen 650 mg PO/PR/IV q6h scheduled (also shivering suppression during TTM rewarm) PO/PR/IV q6h scheduled (antipyretic_analgesic, first line) — Standard analgesia / antipyresis + shivering suppression component; QT-neutral and catecholamine-neutral
15. buspirone 30 mg PO BID for shivering suppression (off-label TTM adjunct per BAIR-Hugger / shivering-control protocols) PO BID (serotonin_5ht1a_partial_agonist, add on) — Serotonergic shivering suppression off-label TTM adjunct; non-catecholaminergic; Choi-style anti-shivering ladder
16. lidocaine 1–1.5 mg/kg IV bolus then 1–4 mg/min infusion bridge for refractory polymorphic VT during arrest pathway only IV continuous bridge (class_ib_sodium_channel_blocker, rescue) — AHA 2020 ACLS standard polymorphic VT bridge; acceptable in CPVT (unlike Brugada where contraindicated); β-blocker + flecainide preferred long-term

Setting playbook (outpatient) — Long-term EP / inherited-arrhythmia clinic surveillance; ICD interrogation q3–6 mo; nadolol maintenance + dose optimization (weight-based in growing pediatric); flecainide adjunct + level monitoring; LCSD for refractory CPVT or β-blocker intolerance or ICD-shock burden; family cascade testing completion + ongoing identification of newly recognised relatives (exercise stress test for relatives); LIFELONG drug-avoidance + sports-avoidance education + lifestyle modifications; annual mental health screen
17. continue nadolol maintenance lifelong (preferred CPVT β-blocker) nadolol 1.5–2.0 mg/kg/d (weight-based titration in growing pediatric) PO daily; lifelong — CPVT diagnosis (HRS 2017 Class I + Roston/Mazzanti registry)
18. continue flecainide adjunct lifelong if on therapy 100–300 mg/d PO BID (target trough 0.2–1.0 µg/mL) PO BID; lifelong — high-risk CPVT or recurrent events on nadolol alone (HRS 2017 IIa + van der Werf JACC 2011)
19. continue lifelong avoidance of sympathomimetics + amphetamines + cocaine + MDMA + high-dose caffeine patient education + curated counseling n/a lifelong — CPVT diagnosis (HRS 2017)

Non-pharmacologic actions:
- EP / inherited-arrhythmia clinic q3–6 mo lifelong
- ICD generator / lead surveillance per device clinic; replacement at end-of-service
- LCSD (left cardiac sympathetic denervation) referral for refractory CPVT despite optimal β-blocker + flecainide, or β-blocker intolerance, or ICD-shock burden (HRS 2017 Class IIa — most effective non-pharmacologic intervention)
- Family cascade testing — ongoing identification of newly recognised relatives (extended pedigree); exercise stress test for relatives is highest yield
- Lifestyle: LIFELONG AVOIDANCE OF COMPETITIVE SPORTS (HRS 2017 Class I); recreational low-intensity activity titrated to symptoms; emotion / stress management techniques; lifelong avoidance of sympathomimetics + ADHD stimulants (substitute non-stimulant atomoxetine if treatment needed) + cocaine + amphetamines + MDMA + high-dose caffeine
- Medic-alert bracelet maintenance — "CPVT — AVOID catecholamines + competitive sports"
- Mental health long-term

AVOID / contraindication checks:
- Isoproterenol_AVOID_in_cpvt (CONTRAINDICATED — β 1 catecholamine directly triggers RyR2 leak; opposite pharmacology vs Brugada where iso is first line storm suppression)
- Epinephrine_chronic_AVOID_in_cpvt (catecholamine surge is THE trigger; AVOID post ROSC infusion if alternative; standard ACLS arrest dose acceptable)
- Dobutamine_AVOID_in_cpvt (β 1 catecholamine inotrope — substitute milrinone for inotropic support)
- High_dose_norepinephrine_AVOID_in_cpvt (minimize dose; substitute vasopressin or phenylephrine when feasible)
- Sympathomimetic_decongestants_pseudoephedrine_AVOID_lifelong_in_cpvt (lifestyle counseling)
- Adhd_stimulants_methylphenidate_dextroamphetamine_AVOID_lifelong_in_cpvt (substitute non stimulant atomoxetine if treatment needed)
- Cocaine_amphetamine_mdma_AVOID_lifelong_in_cpvt (illicit sympathomimetics — direct triggers)
- Caffeine_high_dose_energy_drinks_AVOID_in_cpvt (moderate evidence trigger — counsel limitation)
- Competitive_sports_AVOID_lifelong_in_cpvt (HRS 2017 Class I; allow recreational low intensity activity titrated to symptoms)
- Digoxin_AVOID_in_cpvt (also causes bidirectional VT; mimic + may worsen substrate)
- Beta_blocker_LIFELONG_FIRST_LINE_in_cpvt (nadolol preferred over propranolol or metoprolol per Roston/Mazzanti registry meta analysis)
- Flecainide_ADJUNCT_TO_BB_in_cpvt (van der Werf JACC 2011 + RyR2 mechanism Watanabe Nat Med 2009 — HRS 2017 IIa)
- Icd_required_HRS_2017_class_i_post_cpvt_arrest (sustained VT/VF survivor; program with long detection windows + ATP first to avoid shock storm catecholamine surge)
- Lcsd_for_refractory_cpvt_HRS_2017_class_iia (left cardiac sympathetic denervation — most effective non pharmacologic intervention)
- Exercise_stress_ecg_diagnostic_gold_standard_NOT_resting_ecg (resting ECG is normal in CPVT)

Monitoring

Regimen monitoring:
- continuous ecg telemetry x 48-72h with bidirectional polymorphic vt surveillance (HRS 2017)
- serial ecg q4-6h x 24h then q6-8h x 48h (document baseline normalcy + post-storm absence of arrhythmia)
- BMP q4-6h until K above 4 and Mg above 2 sustained (supportive)
- 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 + buspirone + adequate sedation — shivering = catecholamine surge trigger)
- 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)
- daily medication audit for sympathomimetics (lifelong drug avoidance)
- genetic panel ryr2 casq2 core then expanded if negative (HRS 2017)
- cardiac MRI at 4-6 wk (rule out ARVC overlap)
- EXERCISE STRESS ECG at inherited arrhythmia center post stabilization (gold-standard diagnostic — NOT resting ECG)
- first degree relative ECG AND EXERCISE STRESS TEST screening referral (cascade testing — exercise test is highest yield)
- icd interrogation q3 to 6 mo program long detection atp first (avoid shock-storm catecholamine surge)

Setting (outpatient) monitoring:
- q3–6 mo ICD interrogation lifelong
- Annual ECG
- Annual exercise stress test under EP supervision
- Annual Holter if β-blocker titration or symptoms
- Annual flecainide level if on therapy
- Family cascade testing progress documentation
- Lifelong drug + sports audit at every clinic visit

Follow-up plan: Cardiology + EP / inherited-arrhythmia clinic at 2–4 wks; cardiac MRI at 4–6 wk (rule out ARVC overlap; allow post-stunning resolution); EXERCISE STRESS ECG at inherited-arrhythmia center post-stabilization (treadmill or bicycle with continuous ECG + defibrillator pads + EP supervision — diagnostic gold standard for CPVT, ~75% sensitivity even with normal resting ECG); GENETIC PANEL completed (RYR2/CASQ2 core; expanded if needed); CASCADE FAMILY SCREENING — first-degree relatives ECG + EXERCISE STRESS TEST (highest yield) + genotyping at proband mutation; nadolol maintenance + flecainide adjunct; ICD interrogation q3–6 mo; LCSD evaluation for refractory CPVT or β-blocker intolerance; LIFELONG AVOIDANCE of competitive sports (HRS 2017 Class I); recreational low-intensity activity titrated to symptoms; emotion / stress trigger management; lifelong avoidance of sympathomimetics + amphetamines + cocaine + MDMA + high-dose caffeine; medic-alert bracelet "CPVT — AVOID catecholamines + competitive sports"; PTSD / mental health screen
- Close-out criterion: cardiology + EP follow-up + exercise stress ECG + genetic panel + cascade family screening + ICD + nadolol + flecainide adjunct + LCSD evaluation + competitive-sports avoidance + lifestyle modifications + mental health all booked / documented

Monitoring phase: Continuous telemetry × 48–72 h with bidirectional / polymorphic VT surveillance; A-line; multimodal neuroprog ≥72 h post-rewarm (Sandroni 2021 PMID 33745427); aggressive shivering suppression during TTM rewarm; serial ECG q4–6 h × 24 h then q6–8 h × 48 h documenting baseline normalcy; daily medication audit for sympathomimetics; nadolol initiation logging once storm controlled

Disposition

Current setting: outpatient — Long-term EP / inherited-arrhythmia clinic surveillance; ICD interrogation q3–6 mo; nadolol maintenance + dose optimization (weight-based in growing pediatric); flecainide adjunct + level monitoring; LCSD for refractory CPVT or β-blocker intolerance or ICD-shock burden; family cascade testing completion + ongoing identification of newly recognised relatives (exercise stress test for relatives); LIFELONG drug-avoidance + sports-avoidance education + lifestyle modifications; annual mental health screen

Disposition criteria:
- Long-term continuation lifelong; ICD + nadolol + flecainide adjunct if indicated + LCSD if refractory + lifelong drug avoidance + lifelong sports avoidance + family cascade testing operational

Escalation triggers (move to higher acuity):
- Recurrent ICD shock → emergent EP + storm investigation; LCSD evaluation expedited
- New sympathomimetic drug exposure → ED + drug withdrawal + reassessment
- Family member positive screening on exercise stress test → cascade testing extended + EP referral
- Nadolol intolerance → LCSD evaluation; alternate β-blocker; flecainide dose optimization
- Mental health crisis → psychiatry
- Sports / activity non-compliance → reinforce education + family meeting + EP counseling

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Recurrent bidirectional / polymorphic VT or VF post-ROSC suggests ongoing CPVT storm physiology — esmolol IV bridge + magnesium + flecainide PO load + urgent EP for LCSD evaluation; AVOID isoproterenol (CONTRAINDICATED — directly triggers RyR2 leak); minimize epinephrine + NE
- [SEVERE] Inadvertent administration of catecholamine drug post-ROSC (isoproterenol, dobutamine, high-dose epinephrine infusion, sympathomimetic decongestant, amphetamine) in confirmed or suspected CPVT — STOP drug + escalate to EP + chart audit + nursing handoff review; substitute with catecholamine-sparing alternative (vasopressin, phenylephrine, milrinone)
- [SEVERE] Shivering during TTM rewarm in CPVT post-arrest is a catecholamine surge trigger — aggressive shivering control with magnesium + acetaminophen + buspirone + adequate sedation; if refractory, propofol bolus rescue + neuromuscular blockade (last-line); slow rewarm 0.25–0.5 °C/h

Citations

- HRS 2017 Inherited Arrhythmia Syndromes Expert Consensus (Al-Khatib PMID 28219760) + AHA 2020 ACLS / Post-Cardiac-Arrest Care + TTM2 + Sandroni 2021 ERC-ESICM neuroprog + Roston / Mazzanti CPVT registry meta-analysis (nadolol superior β-blocker) + van der Werf JACC 2011 (flecainide adjunct) + Watanabe Nat Med 2009 (flecainide RyR2 mechanism) + Wilde 2008 (LCSD efficacy) + ESC 2022 VA / SCD prevention [PMID:28219760](https://pubmed.ncbi.nlm.nih.gov/28219760/)
- Cited evidence (PMID 27406239) [PMID:27406239](https://pubmed.ncbi.nlm.nih.gov/27406239/)
- Cited evidence (PMID 29804673) [PMID:29804673](https://pubmed.ncbi.nlm.nih.gov/29804673/)
- Cited evidence (PMID 21962432) [PMID:21962432](https://pubmed.ncbi.nlm.nih.gov/21962432/)
- Cited evidence (PMID 19620991) [PMID:19620991](https://pubmed.ncbi.nlm.nih.gov/19620991/)

Last reconciled with current guidelines: 2026-05-15.
References
  • HRS 2017 Inherited Arrhythmia Syndromes Expert Consensus (Al-Khatib PMID 28219760) + AHA 2020 ACLS / Post-Cardiac-Arrest Care + TTM2 + Sandroni 2021 ERC-ESICM neuroprog + Roston / Mazzanti CPVT registry meta-analysis (nadolol superior β-blocker) + van der Werf JACC 2011 (flecainide adjunct) + Watanabe Nat Med 2009 (flecainide RyR2 mechanism) + Wilde 2008 (LCSD efficacy) + ESC 2022 VA / SCD prevention — PMID:28219760
  • Cited evidence (PMID 27406239) — PMID:27406239
  • Cited evidence (PMID 29804673) — PMID:29804673
  • Cited evidence (PMID 21962432) — PMID:21962432
  • Cited evidence (PMID 19620991) — PMID:19620991