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cardio.cardiogenic-shock.brugada-storm.v1

Cardiogenic shock — Brugada syndrome electrical storm

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Phase E variant of cardio.cardiogenic-shock.core.v1 — narrowed to Brugada syndrome electrical storm (≥3 sustained polymorphic VT / VF episodes in 24 h or incessant VF) with hemodynamic collapse / cardiogenic shock. SCN5A loss-of-function (~20–30% of Brugada) → reduced cardiac Na+ current (INa) → transmural voltage gradient in RV outflow tract → Type 1 Brugada ECG (V1–V3 coved ST ≥2 mm + T-wave inversion) and substrate for phase-2 reentry → polymorphic VT/VF. Per HRS 2017 PMID 28219760. Treatment pivots from standard CS bundle: ISOPROTERENOL infusion 1–3 µg/min FIRST-LINE (paradoxical β-1 agonist that suppresses storm by augmenting L-type Ca current ICa-L); QUINIDINE 1–2 g/d PO loading then 600–1200 mg/d maintenance (the ONLY Na-channel blocker that helps in Brugada because it also blocks Ito reducing phase-2 dispersion — Belhassen registry); aggressive antipyresis (fever is the MOST COMMON modifiable trigger); STOP all Na-channel-blocking drugs (www.brugadadrugs.org curated list); K ≥4.5 + Mg ≥2.0 repletion; ICD shocks for sustained VF; cautious NE for MAP support. AVOID (paradoxical / harmful in Brugada storm): lidocaine, procainamide, flecainide, propafenone (all Na-channel blockers — lethal at therapeutic dose; diagnostic at low dose for Brugada pattern unmasking); amiodarone (variable / unproven benefit; can prolong QT further); β-blockers (variable; may worsen storm by removing β-1 stabilization — opposite of LQT pharmacology). Refractory storm → catheter ablation of RVOT epicardial substrate per Nademanee 2011 PMID 21571989 (suppresses storm in 75–80% of refractory cases). Long-term ICD policy per HRS 2017: Class I for sustained VT/VF survivors or syncope + spontaneous Type 1 ECG; Class IIa for asymptomatic Type 1 + family history of sudden death <45 y; Class III for drug-induced Type 1 alone in asymptomatic patient. Cascade testing of first-degree relatives + SCN5A genetic panel offered. Lifelong drug avoidance + medic-alert bracelet + aggressive fever management mandatory. Inherits parent CS framework (vasopressor / MCS escalation, MDT activation); specialises for Brugada-specific pharmacology (isoproterenol FIRST-LINE storm suppression, quinidine Ito blockade, AVOID all other Na-channel blockers, aggressive trigger reversal, RVOT substrate ablation pathway). Status INTEGRATED until terminology + RxNav-validated drug codes are reconciled. Authored 2026-05-15 by shard-06-cardio-acute as Phase E wave 16 channelopathy storm variant.

Entry points (5)

  • imaging
    ECG Type 1 Brugada (V1–V3 coved ST elevation ≥2 mm + T-wave inversion) + sustained polymorphic VT / VF episodes — electrical storm with shock physiology
    ecg_type1_brugada_pattern_with_storm
  • symptom
    Recurrent ICD shocks (≥3 in 24 h) in known Brugada patient — electrical storm; assess for fever / Na-channel-blocker exposure trigger
    recurrent_icd_shocks_in_brugada_patient
  • history
    Syncope or aborted SCD in patient with spontaneous or drug-induced Type 1 Brugada ECG; family history of sudden death <45 y
    syncope_with_type1_brugada_ecg
  • history
    Fever (T >38°C) precipitating polymorphic VT in known Brugada patient — most common storm trigger
    fever_triggered_polymorphic_vt_in_known_brugada
  • history
    Inadvertent administration of Na-channel-blocker (lidocaine, procainamide, flecainide, propafenone, cocaine) precipitating storm — STOP drug + reverse with isoproterenol
    inadvertent_na_channel_blocker_exposure_in_brugada

Required inputs (14)

  • agerequired
    demographic • used at CONTEXT
    Brugada most often manifests age 30–50 y, male predominance ~8:1; informs ICD eligibility decision
  • sexrequired
    demographic • used at CONTEXT
    Male predominance ~8:1 (testosterone effect on Ito); influences risk stratification
  • sbprequired
    vital • used at RED_FLAGS
    SCAI 2022 staging baseline + vasopressor titration; storm with hemodynamic collapse defines CS overlay
  • hrrequired
    vital • used at CONTEXT
    Compensatory tachy / brady + pre-arrest state; influences isoproterenol titration target (HR 90–110 typical target)
  • temperaturerequired
    vital • used at RED_FLAGS
    Fever is the MOST COMMON modifiable trigger of Brugada storm; aggressive antipyresis + ice mandatory
  • lactaterequired
    lab • used at RISK_STRATIFICATION
    SCAI 2022 staging + perfusion; CardShock prognostication (Harjola EHJ 2015 PMID 26333869)
  • creatininerequired
    lab • used at CONTEXT
    End-organ damage marker; eGFR for quinidine dose adjustment
  • potassiumrequired
    lab • used at INITIAL_WORKUP
    Hypokalemia worsens storm (K target ≥4.0); aggressive replacement mandatory
  • magnesiumrequired
    lab • used at INITIAL_WORKUP
    Hypomagnesemia precipitates polymorphic VT; replace to ≥2.0
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Differentiate from ischemic cause of polymorphic VT; usually negative or modest in primary Brugada storm
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    Type 1 Brugada pattern in V1–V3 (coved ST ≥2 mm + T-wave inversion) — diagnostic anchor; serial ECGs to track ST evolution; high V1–V2 lead placement (2nd–3rd ICS) increases sensitivity
  • echorequired
    imaging • used at INITIAL_WORKUP
    STRUCTURALLY NORMAL HEART expected (Brugada is channelopathy, not cardiomyopathy); rules out ischemic / structural cause of polymorphic VT
  • family_history_sudden_death
    history • used at CONTEXT
    Family history <45 y SCD raises ICD threshold; SCN5A mutation panel offered to first-degree relatives
  • recent_drug_exposure_na_channel_blockerrequired
    history • used at CONTEXT
    STOP all Na-channel blockers (lidocaine, procainamide, flecainide, propafenone, cocaine); www.brugadadrugs.org curated avoid-list

12-phase flow (11)

  1. 1FRAME
    Recognize Brugada electrical storm with shock — channelopathy + Type 1 ECG + storm-defining ≥3 VT/VF in 24 h + hemodynamic collapse; this is a primary electrical disease, NOT ischemic
    inputs: ecg, sbp
    advance: Brugada storm with CS overlay confirmed
  2. 2ENTRY
    Activate EP team + CS team; immediate trigger reversal (antipyresis, stop Na-channel blockers, K + Mg repletion); initiate isoproterenol if storm ongoing
    inputs: sbp, temperature
    advance: EP team activated + isoproterenol started if storm active
  3. 3CONTEXT
    Family history of SCD, prior syncope, prior ICD, current medications (Na-channel blocker exposure check), recent fever / illness, alcohol / cocaine use
    inputs: hr, creatinine, recent_drug_exposure_na_channel_blocker, family_history_sudden_death
    advance: Triggers identified + family history documented
  4. 4RED_FLAGS
    Active VF / hemodynamically unstable VT (immediate defibrillation); fever (most common modifiable trigger); inadvertent Na-channel-blocker exposure; severe hypokalemia; pre-arrest physiology; LV dysfunction (think of mimic — structural heart disease changes management)
    inputs: sbp, temperature, potassium
    actions: cardiogenic_shock, wide_complex_tach
    advance: Acute arrhythmias managed + reversible triggers screened
  5. 5INITIAL_WORKUP
    ECG (Type 1 Brugada pattern V1–V3 with high V1–V2 placement at 2nd–3rd ICS for sensitivity); STAT echo (rule out structural disease — Brugada heart is normal); troponin (rule out ischemic cause); BMP + Mg + Ca; CXR; tox screen for cocaine; comprehensive Na-channel blocker drug review
    inputs: ecg, echo, troponin, lactate, potassium, magnesium
    actions: cardiogenic_shock, wide_complex_tach, panel.cardiac, panel.renal
    advance: Brugada confirmed + structural/ischemic causes excluded
  6. 6BRANCHING_WORKUP
    Genetic testing (SCN5A panel; ~20–30% positive); cardiology referral for first-degree relatives screening; emergent angiography ONLY if troponin elevated or ECG suggests OMI overlay (rare)
    inputs: ecg
    actions: acs_pathway
    advance: Genetic / family screening triggered + obstructive CAD ruled out if troponin positive
  7. 7RISK_STRATIFICATION
    Storm severity (≥3 VF in 24 h vs incessant VF); SCAI 2022 stage; HRS 2017 ICD class (Class I if sustained VT/VF survivor or syncope + Type 1; IIa if asymptomatic Type 1 + family history); refractoriness to isoproterenol + quinidine flag
    inputs: sbp, lactate
    advance: Risk + ICD eligibility class assigned
  8. 8TREATMENT
    STORM SUPPRESSION: ISOPROTERENOL infusion 1–3 µg/min (FIRST-LINE β-1 agonist that paradoxically suppresses Brugada storm by augmenting ICa-L); QUINIDINE 1–2 g/d PO load → 600–1200 mg/d maintenance (Ito blocker — only Na-channel blocker that helps in Brugada); aggressive antipyresis (acetaminophen + cooling); STOP all Na-channel-blocking drugs; K + Mg repletion (K ≥4.5, Mg ≥2.0); ICD shocks for sustained VF as needed; cautious NE for shock support (avoid agents that may worsen ECG). REFRACTORY STORM: catheter ablation of RVOT epicardial substrate (Nademanee 2011 PMID 21571989); MCS bridge per SCAI 2022 / DanGer Shock if SCAI D-E
    inputs: sbp, lactate, temperature
    actions: cardiogenic_shock
    advance: Isoproterenol + quinidine + trigger reversal + ICD policy + MCS plan all in place
  9. 9DISPOSITION
    CICU at EP-capable center; advanced HF / EP center transfer for refractory storm needing ablation or MCS; long-term cardiology + EP / inherited-arrhythmia clinic follow-up
    advance: Disposition assigned + EP team owns long-term plan
  10. 10MONITORING
    Continuous telemetry with Brugada lead placement (high V1–V2); A-line; central line; serial ECGs q4–6 h initially; lactate clearance; UOP; isoproterenol titration to target HR 90–110 + storm suppression; quinidine level monitoring (target 2–5 µg/mL)
    inputs: lactate
    actions: panel.cardiac, panel.renal
    advance: Monitoring + dose titration cadence set
  11. 11FOLLOWUP
    EP / inherited-arrhythmia clinic follow-up 1–4 wks; ICD interrogation at 1 wk, 1 mo, then q3 mo; family genetic counseling + first-degree relative ECG screening (consider provocative ajmaline / flecainide test in genetics center ONLY); www.brugadadrugs.org avoid-list patient education; long-term oral quinidine if storm-prone or bridge-to-ablation
    advance: Long-term EP plan + family screening + drug-avoidance education complete