← Back to dossier
Patient handout

Post-cardiac-arrest care — congenital long-QT channelopathy (KCNQ1 / KCNH2 / SCN5A) with TdP-arrest

PRODUCTION

1. Your condition

This handout is for post-cardiac-arrest care — congenital long-qt channelopathy (kcnq1 / kcnh2 / scn5a) with tdp-arrest. Your care team identified this based on: rosc after out-of-hospital vf arrest with known long-qt syndrome (prior diagnosis, prior syncope, family history) or with sentinel post-rosc ecg features (qtc > 500 ms, tdp runs).

Other reasons your team may use this plan: post-rosc 12-lead ecg with qtc > 500 ms (bazett or fridericia) + structurally normal heart on echo — congenital lqt high concern; pivot from generic post-arrest care to channelopathy-specific avoidance protocol; witnessed arrest with classic lqt trigger pattern: exertion or swimming (lqt1), auditory startle / emotional / post-partum (lqt2), sleep / nocturnal (lqt3) — directs subtype classification; family history of sudden death <40 y or known lqts in first-degree relative — congenital lqt high pretest probability; offer genetic panel + cascade screening.

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
magnesium sulfate2 g IV bolus over 5–15 min then 2 g/h infusion REGARDLESS of measured Mg level; aggressive replacement to Mg ≥2.0IVcontinuous; titrate to TdP suppressionAHA 2020 ACLS — FIRST-LINE for TdP regardless of measured Mg level; mechanism stabilizes myocardial membrane independent of measured Mg; HRS 2017 PMID 28219760
potassium chloride20–40 mEq IV/PO until K ≥4.5 mandatory in LQTIV/POPRN until target sustainedHypokalemia is major TdP precipitant; K ≥4.5 target mandatory in LQT; HRS 2017
norepinephrine0.05–0.5 µg/kg/min titrate MAP ≥65; CAUTION in LQT1/2 (adrenergic trigger — minimize dose to lowest effective)IVcontinuousSOAP-II PMID 20200382; first-line post-ROSC vasoactive; in LQT1/LQT2 use lowest effective dose because adrenergic surge is the trigger for these subtypes
epinephrine1 mg IV q3–5 min during arrestIVstandard ACLSAHA 2020 ACLS — standard arrest pathway; in confirmed LQT post-ROSC minimize subsequent infusions because adrenergic load triggers LQT1/2
propofol5–50 µg/kg/min; titrate RASSIVcontinuousPADIS 2018; preferred sedative in LQT post-arrest because does NOT prolong QT (vs avoiding antipsychotics which do)
fentanyl25–200 µg/hIVcontinuousPADIS 2018; preferred opioid (does NOT prolong QT) over methadone (QT-prolonging — AVOID)
dexmedetomidine0.2–1.4 µg/kg/h; no bolusIVcontinuousPADIS 2018; preferred for ICU delirium in LQT cohort because does NOT prolong QT (vs haloperidol — AVOID); Class IIa AHA delirium prevention
nadololCONGENITAL LQT1/LQT2 long-term: nadolol 1–1.5 mg/kg/d daily (preferred over propranolol per recent registry data — long half-life + better adherence)POdaily; lifelongHRS 2017 PMID 28219760 Class I + Schwartz International LQTS Registry — mortality reduction in LQT1/2 with long-term β-blocker; nadolol preferred over propranolol (long half-life, daily dosing improves adherence)
propranololCONGENITAL LQT1/LQT2 long-term alternate: propranolol 2–4 mg/kg/d divided BID-QIDPOBID-QID; lifelongHRS 2017 Class I; Schwartz registry — historical first-line; QID dosing reduces adherence vs nadolol daily
mexiletineCONGENITAL LQT3 long-term: mexiletine 150–300 mg PO q8hPOTIDHRS 2017 IIa — mexiletine shortens QT in LQT3 by blocking late INa (the gain-of-function defect); does NOT replace ICD
isoproterenol0.5–2 µg/min IV titrate to HR 90–110 for bradycardia-dependent TdP recurrence (LQT3 + acquired); CONTRAINDICATED in LQT1/2IVcontinuousAHA 2020 ACLS — increases HR shortens QT prevents R-on-T; useful for bradycardia-dependent TdP (LQT3 + acquired); CONTRAINDICATED in LQT1/2 (adrenergic trigger)
acetaminophen650 mg PO/PR/IV q6h PRNPO/PR/IVq6h PRNStandard analgesia / antipyresis; does NOT prolong QT

Plan: Congenital LQT post-arrest phenotype — standard post-ROSC bundle + electrolyte optimization + drug-avoidance protocol + lifelong β-blocker initiation + ICD pathway (HRS 2017 Class I) + cascade family screening

3. When to call your provider

Contact your care team if any of the following happen:

  • Recurrent ICD shock → emergent EP + storm investigation; LCSD evaluation
  • New QT-prolonging drug exposure → ED + drug withdrawal + reassessment
  • Family member positive screening → cascade testing extended + EP referral
  • BB intolerance → LCSD evaluation; alternate BB; mexiletine consideration
  • Mental health crisis → psychiatry

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Recurrent TdP / polymorphic VT post-ROSC suggests ongoing storm physiology — MgSO4 2 g IV bolus + 2 g/h infusion regardless of measured Mg level; route to sister cardio.cardiogenic-shock.lqt-tdp-storm.v1 for mid-storm hemodynamic + electrical management; AVOID Class IA + III antiarrhythmics(life-threatening)
  • Inadvertent administration of QT-prolonging drug post-ROSC (haloperidol, ondansetron > 16 mg, citalopram, macrolide, fluoroquinolone, methadone, Class IA/III antiarrhythmic, antipsychotic) in confirmed or suspected LQT — STOP drug + escalate to EP + chart audit + nursing handoff review; substitute with QT-neutral alternative
  • Bradycardia-dependent TdP recurrence post-ROSC (LQT3 phenotype or acquired with high-grade AV block / sinus arrest / post-cardioversion pause) — isoproterenol 0.5–2 µg/min OR overdrive transcutaneous / transvenous pacing at 80–100 bpm; AVOID β-blockers acutely (long-term lifesaving in LQT1/2 only)(life-threatening)
  • Sustained VT/VF survivor by definition meets HRS 2017 Class I ICD criteria — implant pre-discharge or schedule within 1 wk; subcutaneous ICD considered if no pacing indication; WCD bridge if ICD deferred for stabilization
  • Confirmed congenital LQT (or strongly suspected pending genetic results) triggers mandatory cascade screening of first-degree relatives — ECG + genetic testing at proband mutation; genetic counseling referral; many newly identified relatives are asymptomatic carriers requiring lifelong management

5. Follow-up

Cardiology + EP / inherited-arrhythmia clinic at 2–4 wks; cardiac MRI at 4–6 wk (post-stunning resolution); GENETIC PANEL completed (KCNQ1/KCNH2/SCN5A core; expanded if needed); CASCADE FAMILY SCREENING — first-degree relatives ECG + genotyping at proband mutation; lifelong β-blocker (nadolol preferred); ICD interrogation q3–6 mo; LCSD evaluation for shock burden; lifelong drug avoidance (medic-alert bracelet "Long QT — AVOID QT-prolonging drugs"); LQT1 avoid swimming/diving; LQT2 avoid sudden loud noises (alarm clock modification); LQT3 caution sleep alone + nocturnal monitoring; aggressive K repletion if hypokalemic; PTSD / mental health screen

6. Sources

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 + Schwartz International LQTS Registry + ESC 2022 VA / SCD prevention + CredibleMeds (www.crediblemeds.org) curated QT-prolonging drug list (gold standard reference)

  1. pubmed.ncbi.nlm.nih.gov/28219760
  2. pubmed.ncbi.nlm.nih.gov/33098585
  3. pubmed.ncbi.nlm.nih.gov/33081530