Cardiogenic shock — giant cell myocarditis (GCM)
Encounter flow
11/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
Suspect GCM in previously healthy young-to-middle-aged adult with rapid progression of acute HF to CS, especially with sustained VT/VF storm or new high-grade AV block; EMB is the gateway diagnostic — multidisciplinary alignment for emergent biopsy
GCM clinical suspicion stated and EMB pathway engaged
Patient inputs (18)
Mandatory rule-out of obstructive CAD (myocarditis is an MI mimic); typically clean coronaries in GCM
Lake Louise Criteria 2018 (Ferreira PMID 30025572); patchy or extensive transmural LGE possible in GCM
Hilar / mediastinal lymphadenopathy screen for sarcoidosis differential; thymoma screen for paraneoplastic GCM
GOLD STANDARD per AHA/ACC/ESC 2007 EMB consensus (Cooper PMID 17998456) — Class IIa for unexplained new-onset HF <2 wks with hemodynamic compromise + ventricular arrhythmia or AV block; multinucleated giant cells + lymphocyte infiltrate + myocyte necrosis WITHOUT non-caseating granulomas (vs sarcoidosis)
GCM mean age ~42-43 yo per Cooper 1997 + Kandolin 2013; younger than typical AMI-CS; informs prognosis + transplant candidacy
Sustained VT/VF storm + AV block both common in GCM; informs telemetry and arrhythmia management
End-organ damage marker + cyclosporine renal dose adjustment + gadolinium contrast safety for CMR
Markedly elevated typical; persistent elevation reflects ongoing myocyte injury despite immunosuppression
Acute HF marker; trend tracks LV recovery on combined immunosuppression
Baseline cytopenias + ATG / cyclosporine + steroid bone marrow surveillance
Cyclosporine + ATG hepatotoxicity surveillance; baseline before immunosuppression
Biventricular dysfunction; effusion screen; no regional wall motion crossing single coronary territory
AV block (15-50% of GCM cases) — high diagnostic value; diffuse ST/T-wave changes
SCAI 2022 staging baseline; gates vasopressor escalation
SCAI 2022 staging + CardShock prognostication (Harjola EHJ 2015 PMID 26333869)
Autoimmune thyroid disease association with GCM (Cooper 1997)
Autoimmune disease screen; thymoma association
Metabolic activity assessment; differential vs cardiac sarcoidosis (Birnie HRS 2014 PMID 24682272)
* = hard-required. Engine cannot meaningfully run until these are filled.
Severity triggers (5)
- informationallife_threateningrecurrent_vt_vf_storm_requiring_icd_in_gcmRecurrent VT/VF storm in GCM (50-60% of cases per Cooper 1997 + Kandolin 2013) — amiodarone + EP consult + ICD implantation given high recurrent arrhythmia risk; refractory cases → catheter ablation; transplant escalationTrigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningtransplant_listing_priority_unos_1a_in_gcmGCM with refractory CS on optimal therapy OR refractory VT/VF on optimal therapy OR MCS-dependent — UNOS 1A transplant listing priority; high recurrence in graft (20-25%) but transplant remains best option given dismal medical mortalityTrigger could not be auto-evaluated — needs clinician judgement.
- informationallife_threateningmcs_bridge_to_immunosuppression_response_in_gcmRefractory CS in GCM despite cautious vasopressor / inotrope — escalate to MCS (IABP / Impella / VA-ECMO) early; bridge to combined immunosuppression response, recovery, or transplant; recovery possible with combined immunosuppression in 40-50% per Cooper GIANT-2Trigger could not be auto-evaluated — needs clinician judgement.
- informationalseverehigh_grade_av_block_requiring_permanent_pacing_in_gcmNew high-grade AV block (Mobitz II / complete heart block) in GCM (15-50% of cases per Kandolin 2013) — transvenous pacemaker → permanent pacemaker (often biventricular if HFrEF); AV block is the major diagnostic clue distinguishing GCM from viral myocarditisTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseveregraft_gcm_recurrence_after_transplantGCM recurrence in transplanted graft on surveillance EMB (20-25% per Kandolin 2013 + Ekström 2024) — re-escalate immunosuppression intensity (steroid pulse + cyclosporine trough increase + add MMF if not already + consider abatacept/ATG re-induction); selected patients may require re-transplantTrigger could not be auto-evaluated — needs clinician judgement.
Workflow calculators
Run this disease's risk and dosing calculators inline.
Recommended regimen
Giant cell myocarditis CS — combined immunosuppression per Cooper GIANT-2 protocol (methylprednisolone + cyclosporine + IL-2RA + ATG induction; chronic maintenance with cyclosporine + low-dose steroids + AZA/MMF) + early MCS bridge + transplant listing priority- norepinephrinefirst linevasopressor_alpha0.05–0.5 µg/kg/min titrate MAP ≥65 • IV • continuoustriggers: gcm_cs_with_sbp_lt_90, cs_scai_c_or_higherSOAP-II PMID 20200382 — NE first-line in CSrxcui 7512
- dobutaminesecond lineinotrope_beta12.5 µg/kg/min CAUTIOUS titration; AVOID in ventricular electrical storm • IV • continuoustriggers: low_cardiac_output_despite_NE, no_active_VT_or_VFDOREMI PMID 33704937; CAUTION in GCM given high VT/VF risk; escalate to MCS over higher inotrope dosesrxcui 3616
- methylprednisolonefirst linecorticosteroid_systemic1000 mg IV daily × 3-5 days then prednisone 1 mg/kg/d taper over months • IV • dailytriggers: gcm_emb_confirmed_or_strongly_suspectedCooper Multicenter GCM Registry NEJM 1997 (PMID 9197214) + GIANT-2 protocol (PMID 18369191) — cornerstone of combined immunosuppressionrxcui 6902
- cyclosporinefirst linecalcineurin_inhibitor3-5 mg/kg/d divided BID; trough target 200-300 ng/mL acute then 100-200 ng/mL maintenance • PO/IV • BIDtriggers: gcm_emb_confirmedCooper NEJM 1997 + GIANT-2 — combination cyclosporine + steroids markedly improves transplant-free survival from ~10% to ~70%; ESC 2013 myocarditis position paperrxcui 3008
- basiliximabadd onil2_receptor_antagonist20 mg IV day 0 + day 4 (induction) • IV • q4d × 2 dosestriggers: gcm_emb_confirmed_severe, transplant_listedGIANT-2 protocol PMID 18369191 — IL-2RA induction in GCM; transplant immunology precedent (basiliximab is standard induction in solid organ transplant)rxcui 196102
- rabbit anti-thymocyte globulinadd onpolyclonal_anti_t_cell1.5 mg/kg/d IV × 5-14 days (induction); replaces historical OKT3 (muromonab) which is no longer available • IV • daily × 5-14 dtriggers: gcm_severe_emb_confirmed, transplant_listed, ventricular_arrhythmia_storm_in_gcmCooper GIANT-2 protocol PMID 18369191 — anti-T-cell induction in GCM; historically OKT3 (muromonab) used but no longer manufactured; ATG is contemporary equivalentrxcui 107044
- azathioprineadd onantimetabolite_purine1-2 mg/kg/d PO daily (chronic maintenance) • PO • dailytriggers: gcm_chronic_maintenance_phaseGIANT-2 protocol PMID 18369191 — chronic maintenance after acute phase; purine antimetaboliterxcui 1256
- mycophenolate mofetiladd onantimetabolite_imp_dh1000 mg PO BID (chronic maintenance alternative to AZA) • PO • BIDtriggers: gcm_chronic_maintenance_alternative_to_aza, aza_intolerantSolid organ transplant precedent + GIANT-2 — alternative to AZArxcui 68149
- amiodaronefirst lineclass_iii_antiarrhythmic150 mg IV bolus then 1 mg/min × 6 h then 0.5 mg/min • IV • continuoustriggers: ventricular_electrical_storm_in_gcm, sustained_vt_or_vfAHA 2020 ACLS Class IIb for refractory VT/VF; high baseline arrhythmia risk in GCM (50-60%); consider catheter ablation if refractoryrxcui 703
- lidocaineadd onclass_ib_antiarrhythmic1-1.5 mg/kg IV bolus then 1-4 mg/min infusion • IV • continuoustriggers: vt_vf_refractory_to_amiodaroneAHA 2020 ACLS alternative for refractory VT/VFrxcui 6387
- magnesium sulfateadd onelectrolyte_repletion2 g IV bolus over 5-15 min then maintenance to keep Mg ≥2.0 • IV • as neededtriggers: vt_vf_torsades, mg_lt_2AHA 2020 ACLS for torsades; standard electrolyte repletionrxcui 6585
- sulfamethoxazole-trimethoprimadd onpjp_prophylaxis1 SS tab PO daily or 1 DS tab PO MWF • PO • daily/MWFtriggers: chronic_immunosuppression_in_gcmPJP prophylaxis on chronic high-dose steroid + cyclosporine; standard transplant immunologyrxcui 10831
- valgancicloviradd oncmv_prophylaxis900 mg PO daily × 3-6 mo per CMV serostatus • PO • dailytriggers: gcm_post_transplant, high_intensity_immunosuppressionCMV prophylaxis on ATG / IL-2RA / chronic immunosuppression; standard transplant immunologyrxcui 275891
outpatient playbook — drug actions (5)
- 1. continue or up-titrate GDMT 4-pillar if persistent EF<40lisinopril or sac/val + carvedilol + MRA + SGLT2i • PO • daily/BIDtrigger: Persistent EF<40AHA/ACC/HFSA 2022 HF Guideline (PMID 35363499)
- 2. continue combined immunosuppression LIFE-LONG OR until transplantrxcui 3008cyclosporine trough 100-200 ng/mL chronic; prednisone 5-10 mg/d; AZA / MMF maintenance • PO • dailytrigger: GCM chronic maintenance / post-transplantCooper NEJM 1997 — chronic combined immunosuppression maintains transplant-free survival; recurrence in graft 20-25% per Kandolin 2013 + Ekström 2024
- 3. continue opportunistic prophylaxisrxcui 8163TMP-SMX + valganciclovir per intensity • PO • dailytrigger: Chronic immunosuppressionStandard transplant immunology
- 4. AVOID NSAIDs lifelongdo not give • n/a • n/atrigger: GCM historyESC 2013
- 5. AVOID live vaccines lifelong on immunosuppressioninactivated only • n/a • n/atrigger: Chronic immunosuppressionStandard transplant immunology
Auto-drafted A&P note
outpatientSubjective
- Possible entry pathways: Previously healthy young-to-middle-aged adult with rapid (days-to-weeks) progression of acute HF to cardiogenic shock — high suspicion for GCM, especially if associated with VT/VF storm or AV block; Sustained VT/VF storm (≥3 episodes in 24 h) in setting of acute myocarditis — GCM until proven otherwise (50-60% of GCM cases per Cooper 1997 + Kandolin 2013); emergent EMB indication; New high-grade AV block (Mobitz II / complete heart block) in acute myocarditis — GCM is the leading clue (15-50% per Kandolin 2013); emergent EMB indication.
Objective
- No vitals, labs, or imaging entered for this encounter.
Assessment
**Cardiogenic shock — giant cell myocarditis (GCM)** (cardio.cardiogenic-shock.giant-cell-myocarditis.v1). Scope: Suspect GCM in previously healthy young-to-middle-aged adult with rapid progression of acute HF to CS, especially with sustained VT/VF storm or new high-grade AV block; EMB is the gateway diagnostic — multidisciplinary alignment for emergent biopsy No severity triggers fired against current inputs.
Plan
Regimen axis: **Giant cell myocarditis CS — combined immunosuppression per Cooper GIANT-2 protocol (methylprednisolone + cyclosporine + IL-2RA + ATG induction; chronic maintenance with cyclosporine + low-dose steroids + AZA/MMF) + early MCS bridge + transplant listing priority**. 1. norepinephrine 0.05–0.5 µg/kg/min titrate MAP ≥65 IV continuous (vasopressor_alpha, first line) — SOAP-II PMID 20200382 — NE first-line in CS 2. dobutamine 2.5 µg/kg/min CAUTIOUS titration; AVOID in ventricular electrical storm IV continuous (inotrope_beta1, second line) — DOREMI PMID 33704937; CAUTION in GCM given high VT/VF risk; escalate to MCS over higher inotrope doses 3. methylprednisolone 1000 mg IV daily × 3-5 days then prednisone 1 mg/kg/d taper over months IV daily (corticosteroid_systemic, first line) — Cooper Multicenter GCM Registry NEJM 1997 (PMID 9197214) + GIANT-2 protocol (PMID 18369191) — cornerstone of combined immunosuppression 4. cyclosporine 3-5 mg/kg/d divided BID; trough target 200-300 ng/mL acute then 100-200 ng/mL maintenance PO/IV BID (calcineurin_inhibitor, first line) — Cooper NEJM 1997 + GIANT-2 — combination cyclosporine + steroids markedly improves transplant-free survival from ~10% to ~70%; ESC 2013 myocarditis position paper 5. basiliximab 20 mg IV day 0 + day 4 (induction) IV q4d × 2 doses (il2_receptor_antagonist, add on) — GIANT-2 protocol PMID 18369191 — IL-2RA induction in GCM; transplant immunology precedent (basiliximab is standard induction in solid organ transplant) 6. rabbit anti-thymocyte globulin 1.5 mg/kg/d IV × 5-14 days (induction); replaces historical OKT3 (muromonab) which is no longer available IV daily × 5-14 d (polyclonal_anti_t_cell, add on) — Cooper GIANT-2 protocol PMID 18369191 — anti-T-cell induction in GCM; historically OKT3 (muromonab) used but no longer manufactured; ATG is contemporary equivalent 7. azathioprine 1-2 mg/kg/d PO daily (chronic maintenance) PO daily (antimetabolite_purine, add on) — GIANT-2 protocol PMID 18369191 — chronic maintenance after acute phase; purine antimetabolite 8. mycophenolate mofetil 1000 mg PO BID (chronic maintenance alternative to AZA) PO BID (antimetabolite_imp_dh, add on) — Solid organ transplant precedent + GIANT-2 — alternative to AZA 9. amiodarone 150 mg IV bolus then 1 mg/min × 6 h then 0.5 mg/min IV continuous (class_iii_antiarrhythmic, first line) — AHA 2020 ACLS Class IIb for refractory VT/VF; high baseline arrhythmia risk in GCM (50-60%); consider catheter ablation if refractory 10. lidocaine 1-1.5 mg/kg IV bolus then 1-4 mg/min infusion IV continuous (class_ib_antiarrhythmic, add on) — AHA 2020 ACLS alternative for refractory VT/VF 11. magnesium sulfate 2 g IV bolus over 5-15 min then maintenance to keep Mg ≥2.0 IV as needed (electrolyte_repletion, add on) — AHA 2020 ACLS for torsades; standard electrolyte repletion 12. sulfamethoxazole-trimethoprim 1 SS tab PO daily or 1 DS tab PO MWF PO daily/MWF (pjp_prophylaxis, add on) — PJP prophylaxis on chronic high-dose steroid + cyclosporine; standard transplant immunology 13. valganciclovir 900 mg PO daily × 3-6 mo per CMV serostatus PO daily (cmv_prophylaxis, add on) — CMV prophylaxis on ATG / IL-2RA / chronic immunosuppression; standard transplant immunology Setting playbook (outpatient) — 4-8 wk recovery echo + CMR — confirm LV recovery (variable in GCM, often partial); long-term GDMT if persistent HFrEF; LIFE-LONG combined immunosuppression maintenance; transplant waitlist management or post-transplant immunosuppression with surveillance for graft GCM recurrence (20-25% per Kandolin 2013 + Ekström 2024); EP follow-up — most GCM patients receive ICD given high arrhythmic recurrence risk 14. continue or up-titrate GDMT 4-pillar if persistent EF<40 lisinopril or sac/val + carvedilol + MRA + SGLT2i PO daily/BID — Persistent EF<40 (AHA/ACC/HFSA 2022 HF Guideline (PMID 35363499)) 15. continue combined immunosuppression LIFE-LONG OR until transplant cyclosporine trough 100-200 ng/mL chronic; prednisone 5-10 mg/d; AZA / MMF maintenance PO daily — GCM chronic maintenance / post-transplant (Cooper NEJM 1997 — chronic combined immunosuppression maintains transplant-free survival; recurrence in graft 20-25% per Kandolin 2013 + Ekström 2024) 16. continue opportunistic prophylaxis TMP-SMX + valganciclovir per intensity PO daily — Chronic immunosuppression (Standard transplant immunology) 17. AVOID NSAIDs lifelong do not give n/a n/a — GCM history (ESC 2013) 18. AVOID live vaccines lifelong on immunosuppression inactivated only n/a n/a — Chronic immunosuppression (Standard transplant immunology) Non-pharmacologic actions: - No competitive sports × 6+ mo per AHA 2015 / ESC 2020 / Drezner JACC 2022 (often lifelong restriction in GCM) - Cardiac rehab - EP referral for ICD per AHA 2017 VA/SCD guideline (most GCM patients eligible) - Ongoing transplant waitlist or post-transplant care - Annual flu (inactivated) + indicated inactivated vaccines AVOID / contraindication checks: - Nsaids_AVOID_in_gcm_myocarditis (worsen myocarditis per ESC 2013) - High_dose_dobutamine_minimize_in_gcm (very high VT/VF risk; escalate to MCS instead) - Digoxin_AVOID_in_acute_myocarditis (toxicity threshold lowered per ESC 2013) - Gadolinium_caution_if_egfr_lt_30 (NSF risk; group II macrocyclic agents safer) - Cyclosporine_renal_adjust + trough monitoring 200 300 ng/mL acute then 100 200 ng/mL maintenance (calcineurin nephrotoxicity) - Cyclosporine_drug_interactions (CYP3A4: avoid concurrent strong inhibitors / inducers) - Aza_check_TPMT_genotype_before_initiation (myelosuppression risk if TPMT deficient) - Atg_premedicate_with_acetaminophen_diphenhydramine_methylprednisolone (cytokine release syndrome) - Basiliximab_anaphylaxis_risk (rare but reported; have epinephrine ready) - Opportunistic_infection_prophylaxis_required (PJP, CMV, anti fungal per immunosuppression intensity)
Monitoring
Regimen monitoring: - arterial line continuous BP (ACC/AHA 2022 Class I) - central venous access (ACC/AHA 2022) - lactate q1-2h (CardShock, Harjola EHJ 2015) - UOP hourly (SCAI 2019 end-organ perfusion marker) - serial echo q24h for recovery trajectory - continuous telemetry for arrhythmia (extreme VT/VF risk in GCM) - daily troponin and bnp (trend tracks recovery) - cyclosporine trough q24h (200-300 acute, 100-200 maintenance) - daily CBC LFT BMP for immunosuppression toxicity - CMV PCR weekly during high intensity immunosuppression - PJP prophylaxis TMP-SMX or atovaquone - opportunistic fungal surveillance if neutropenic Setting (outpatient) monitoring: - Echo at 6 mo and 12 mo - CMR at 6 mo if persistent LGE - Surveillance EMB per protocol (some centers q3-6 mo for first year) - Cyclosporine trough monthly - CBC + LFT + BMP + lipid + HbA1c monthly initially - Skin cancer surveillance (chronic immunosuppression risk) Follow-up plan: Repeat echo + CMR at 4-8 wks; continued combined immunosuppression LIFE-LONG OR until transplant; transplant evaluation completion if not yet listed; cardiac rehab; EP follow-up for ICD eligibility per AHA 2017 VA/SCD guideline (high arrhythmic recurrence — most GCM patients receive ICD after acute phase); post-transplant immunosuppression maintained with surveillance for graft GCM recurrence (20-25% per Kandolin 2013 + Ekström 2024) - Close-out criterion: Recovery echo, CMR, continued immunosuppression, transplant evaluation, ICD pathway booked Monitoring phase: A-line, central line, lactate clearance, urine output; continuous telemetry (high VT/VF risk); serial echo q24h for LV recovery; daily troponin and BNP; cyclosporine trough q24h to maintain 200-300 ng/mL acute then 100-200 ng/mL maintenance; daily CBC + LFT for immunosuppression toxicity; opportunistic infection surveillance (CMV, PJP prophylaxis)
Disposition
Current setting: outpatient — 4-8 wk recovery echo + CMR — confirm LV recovery (variable in GCM, often partial); long-term GDMT if persistent HFrEF; LIFE-LONG combined immunosuppression maintenance; transplant waitlist management or post-transplant immunosuppression with surveillance for graft GCM recurrence (20-25% per Kandolin 2013 + Ekström 2024); EP follow-up — most GCM patients receive ICD given high arrhythmic recurrence risk Disposition criteria: - Stable on chronic immunosuppression + GDMT + opportunistic prophylaxis + ICD if eligible → routine surveillance - Persistent HFrEF beyond 6 mo → transplant escalation if not yet listed Escalation triggers (move to higher acuity): - Sustained VT / syncope / ICD shock → EP urgent consult; transplant escalation - Persistent severe LV dysfunction → expedite transplant - Recurrent GCM on surveillance EMB → escalate immunosuppression OR expedite transplant - Opportunistic infection → ID urgent consult - Post-transplant graft GCM recurrence → re-escalate immunosuppression; consider re-transplant in selected cases - Skin cancer / PTLD → oncology + transplant team
Earlier-Return Triggers
Return-precaution thresholds (watch for): - [LIFE_THREATENING] Recurrent VT/VF storm in GCM (50-60% of cases per Cooper 1997 + Kandolin 2013) — amiodarone + EP consult + ICD implantation given high recurrent arrhythmia risk; refractory cases → catheter ablation; transplant escalation - [LIFE_THREATENING] GCM with refractory CS on optimal therapy OR refractory VT/VF on optimal therapy OR MCS-dependent — UNOS 1A transplant listing priority; high recurrence in graft (20-25%) but transplant remains best option given dismal medical mortality - [LIFE_THREATENING] Refractory CS in GCM despite cautious vasopressor / inotrope — escalate to MCS (IABP / Impella / VA-ECMO) early; bridge to combined immunosuppression response, recovery, or transplant; recovery possible with combined immunosuppression in 40-50% per Cooper GIANT-2
Citations
- Cooper Multicenter Giant Cell Myocarditis Registry NEJM 1997 (PMID 9197214); Cooper GIANT-2 trial protocol (PMID 18369191); Caforio ESC 2013 myocarditis position paper (PMID 23824828); Tschöpe AHA 2020 myocarditis scientific statement (PMID 32200645); Cooper AHA/ACC/ESC 2007 EMB consensus (PMID 17998456); Birnie HRS 2014 cardiac sarcoidosis (PMID 24682272) — differential [PMID:9197214](https://pubmed.ncbi.nlm.nih.gov/9197214/) - Cited evidence (PMID 18369191) [PMID:18369191](https://pubmed.ncbi.nlm.nih.gov/18369191/) - Cited evidence (PMID 17998456) [PMID:17998456](https://pubmed.ncbi.nlm.nih.gov/17998456/) - Cited evidence (PMID 23824828) [PMID:23824828](https://pubmed.ncbi.nlm.nih.gov/23824828/) - Cited evidence (PMID 32200645) [PMID:32200645](https://pubmed.ncbi.nlm.nih.gov/32200645/) Last reconciled with current guidelines: 2026-05-15.
- Cooper Multicenter Giant Cell Myocarditis Registry NEJM 1997 (PMID 9197214); Cooper GIANT-2 trial protocol (PMID 18369191); Caforio ESC 2013 myocarditis position paper (PMID 23824828); Tschöpe AHA 2020 myocarditis scientific statement (PMID 32200645); Cooper AHA/ACC/ESC 2007 EMB consensus (PMID 17998456); Birnie HRS 2014 cardiac sarcoidosis (PMID 24682272) — differential — PMID:9197214
- Cited evidence (PMID 18369191) — PMID:18369191
- Cited evidence (PMID 17998456) — PMID:17998456
- Cited evidence (PMID 23824828) — PMID:23824828
- Cited evidence (PMID 32200645) — PMID:32200645