This handout is for post-cardiac-arrest care — cpvt channelopathy (ryr2 / casq2; exercise- or emotion-triggered bidirectional vt → vf arrest). Your care team identified this based on: 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.
Other reasons your team may use this plan: 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); prior unexplained exercise- or emotion-triggered syncope (vs vasovagal pre-syncope which is provoked by upright posture / pain / heat — distinct mechanism) — strong pretest probability for cpvt; missed-opportunity diagnosis.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| 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 | 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 |
| propranolol | CPVT alternate (nadolol unavailable): propranolol 2–4 mg/kg/d divided BID-QID | PO | BID-QID; lifelong | HRS 2017 Class I; Mazzanti 2018 — historical first-line; less effective than nadolol per Roston meta-analysis; QID dosing reduces adherence |
| 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 | 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 |
| 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 | 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 |
| 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 | Standard polymorphic VT supportive therapy |
| 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 | 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 |
| vasopressin | 0.03 U/min IV fixed dose (catecholamine-sparing alternative) | IV | continuous | VANISH-style catecholamine-sparing alternative; non-adrenergic vasopressor preferred in CPVT to minimize RyR2 trigger load |
| phenylephrine | 0.5–10 µg/kg/min IV titrate MAP | IV | continuous | Pure α1 vasopressor without β-1 stimulation — preferred substitute in CPVT to minimize adrenergic trigger; useful when NE not tolerated |
| 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 | AHA 2020 ACLS — standard arrest pathway; in confirmed CPVT post-ROSC AVOID subsequent infusions because epinephrine is the prototypical CPVT trigger |
| milrinone | 0.125–0.5 µg/kg/min IV (catecholamine-sparing inotrope alternative) | IV | continuous | PDE3 pathway — non-catecholamine inotrope; preferred substitute for dobutamine in CPVT to minimize adrenergic trigger load; vasodilator + inotrope |
| midazolam | 0.02–0.1 mg/kg/h IV; titrate RASS | IV | continuous | PADIS 2018; benzodiazepine for sedation + shivering suppression during TTM rewarm — adequate sedation depth blunts catecholamine surge |
| fentanyl | 25–200 µg/h | IV | continuous | PADIS 2018; preferred opioid for analgesia + shivering suppression during TTM rewarm |
| dexmedetomidine | 0.2–1.4 µg/kg/h; no bolus | IV | continuous | PADIS 2018; α2 agonist actually REDUCES central sympathetic outflow — useful sedation adjunct in CPVT for shivering suppression + delirium prevention |
| acetaminophen | 650 mg PO/PR/IV q6h scheduled (also shivering suppression during TTM rewarm) | PO/PR/IV | q6h scheduled | Standard analgesia / antipyresis + shivering suppression component; QT-neutral and catecholamine-neutral |
| buspirone | 30 mg PO BID for shivering suppression (off-label TTM adjunct per BAIR-Hugger / shivering-control protocols) | PO | BID | Serotonergic shivering suppression off-label TTM adjunct; non-catecholaminergic; Choi-style anti-shivering ladder |
| 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 | AHA 2020 ACLS standard polymorphic VT bridge; acceptable in CPVT (unlike Brugada where contraindicated); β-blocker + flecainide preferred long-term |
Plan: 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
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
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
Guideline: 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