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cardio.cardiogenic-shock.amyloidosis-related.v1

Cardiogenic shock — cardiac amyloidosis (ATTR-CM and AL)

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Phase E variant of cardio.cardiogenic-shock.core.v1 — narrowed to cardiogenic shock from severe cardiac amyloidosis (ATTR-CM or AL); distinct from sister cardio.acute-hf.amyloidosis.v1 (decompensation-not-shock) by SHOCK PHYSIOLOGY focus. Pathophysiology: restrictive cardiomyopathy + low cardiac output + often preserved EF + diastolic dysfunction → low forward flow + diuretic-resistance + autonomic dysfunction; LV unable to recover quickly without disease-modifying therapy onset (months) or transplant. Two etiologic phenotypes: ATTR-CM (wild-type elderly or hereditary variant) — tafamidis 61 mg PO daily (ATTR-ACT PMID 30145930) ± gene silencer (patisiran/vutrisiran/inotersen); AL amyloidosis — oncologic emergency, Dara-CyBorD per ANDROMEDA PMID 34077641 ± autologous HSCT. Treatment ACUTE pivots: low-threshold MCS (IABP preferred initial — small LV cavity); cautious volume; AVOID typical inotropes (often paradoxically worse — worsen LVOT obstruction, ventricular arrhythmia, autonomic instability); norepinephrine first-line; IABP for afterload reduction; VA-ECMO / Impella 5.5 bridge to therapy onset (months) or transplant. AVOID list: DIGOXIN (binds amyloid fibrils → toxicity at low serum levels), BETA-BLOCKERS (autonomic intolerance), DILTIAZEM/non-DHP CCB (binds amyloid fibrils + AV block), large-volume diuresis (preload-dependent), ACEi/ARB (orthostasis). Inherits parent CS framework; specialises for amyloid-CS — phenotype-directed disease-modifying therapy, MCS bridging strategy, oncologic emergency pathway for AL, contraindicated drug stewardship, family ATTRv genetic counseling. Status INTEGRATED until terminology + RxNav-validated drug codes are reconciled. Authored 2026-05-15 by shard-06-cardio-acute as Phase E wave 22 infiltrative cardiac variant.

Entry points (5)

  • symptom
    Cardiogenic shock + LV wall thickness ≥12 mm + no long-standing HTN → cardiac amyloidosis with shock physiology
    cs_with_thick_lv_walls_no_htn_history
  • symptom
    Cardiogenic shock + low-voltage QRS on ECG + thick LV walls (voltage-mass mismatch — pathognomonic amyloid signature)
    cs_with_low_voltage_ecg_thick_walls
  • history
    Known cardiac amyloidosis (ATTR or AL) decompensating from chronic HF to shock physiology (SBP <90, lactate ≥2)
    known_amyloid_with_decompensation_to_shock
  • lab_abnormality
    Monoclonal protein on SPEP/SFLC + shock physiology → AL amyloidosis emergent oncologic workup
    monoclonal_protein_with_shock
  • imaging
    Apical sparing pattern on speckle-tracking strain echo (cherry-on-top sign) in shock — amyloid-CS phenotype
    apical_sparing_strain_with_shock

Required inputs (17)

  • agerequired
    demographic • used at CONTEXT
    ATTRwt overwhelmingly age >65; AL across ages; ATTRv often presents 30–60 with hereditary mutation
  • race_ethnicity
    demographic • used at CONTEXT
    V122I ATTR variant prevalent in West African / African-American descent (~3–4%) — drives variant ATTR screen
  • family_history_neuropathy_or_carpal_tunnel
    history • used at CONTEXT
    ATTRv signal — autonomic + peripheral neuropathy + bilateral carpal tunnel release history
  • sbprequired
    vital • used at RED_FLAGS
    SCAI 2022 staging baseline; amyloid CS often hypotensive at baseline due to autonomic + restrictive physiology — narrow tolerance window
  • hrrequired
    vital • used at RED_FLAGS
    AF very common in amyloid (~70% lifetime); rate control challenging — BB and non-DHP CCB poorly tolerated / contraindicated
  • spo2required
    vital • used at INITIAL_WORKUP
    Pulm congestion from restrictive filling drives congestion easily; intubation may worsen restrictive low-output state
  • lactaterequired
    lab • used at RED_FLAGS
    SCAI staging anchor + amyloid CS often has marked lactic acidosis from restrictive low-output state
  • nt_probnprequired
    lab • used at INITIAL_WORKUP
    Markedly elevated in amyloid (often >3000 even with normal LVEF); Mayo AL staging variable (>1800)
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Persistently elevated in amyloid (myocardial infiltration); Mayo AL staging variable (cTnT >0.025 or hsTnT >40)
  • serum_free_light_chainsrequired
    lab • used at BRANCHING_WORKUP
    MANDATORY before PYP read — rule out AL amyloidosis (PYP can be falsely positive in AL); difference >18 mg/L abnormal (Mayo AL staging)
  • spep_upep_immunofixationrequired
    lab • used at BRANCHING_WORKUP
    Detect monoclonal protein for AL workup (combined sensitivity ~99% with SFLC)
  • creatininerequired
    lab • used at CONTEXT
    Cardiorenal common in amyloid; tafamidis dose unaffected; patisiran requires baseline LFT/eGFR
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    Low-voltage QRS + pseudo-infarct pattern + AV conduction abnormalities; may show pseudo-STEMI mimicking ACS
  • echo_strainrequired
    imaging • used at INITIAL_WORKUP
    Apical sparing pattern (preserved apical longitudinal strain with reduced basal strain — cherry-on-top sign); LV wall thickness + restrictive filling + diastolic dysfunction
  • pyp_scan_or_embrequired
    imaging • used at BRANCHING_WORKUP
    TTR PYP scan: visual grade 2-3 + H/CL ratio ≥1.5 + negative monoclonal screen → ATTR diagnosis (no biopsy needed); otherwise endomyocardial biopsy with mass spec for definitive typing
  • ttr_genotype
    lab • used at BRANCHING_WORKUP
    Distinguish ATTRwt vs ATTRv after ATTR diagnosis confirmed (drives family screening + transplant strategy)
  • transplant_eligibility
    history • used at CONTEXT
    Heart transplant ± combined heart-liver (ATTRv) for selected; advanced HF team early evaluation

12-phase flow (12)

  1. 1FRAME
    Cardiac amyloidosis presenting with shock physiology — distinct from sister acute-hf-amyloidosis variant by hemodynamic compromise; phenotype-first triage (AL = oncologic emergency vs ATTR = disease-modifying therapy with months-to-benefit needing MCS bridge)
    inputs: age, sbp
    advance: Amyloid CS confirmed and AL vs ATTR phenotype routing initiated
  2. 2ENTRY
    CS + thick walls / low voltage ECG / known amyloid decompensating / monoclonal protein → activate cards + advanced HF + hematology (if AL) + cardiology amyloid center; mobilize MCS team early given restrictive low-output state recovery limitation
    inputs: sbp, lactate
    advance: Multi-disciplinary team activated + MCS team aware
  3. 3CONTEXT
    Demographics, family history, neuropathy, prior monoclonal workup, transplant eligibility, baseline renal/hepatic function (KDIGO 2021), prior amyloid-directed therapy
    inputs: age, sbp, creatinine, family_history_neuropathy_or_carpal_tunnel, transplant_eligibility
    advance: Context complete and AL vs ATTR phenotype + transplant eligibility documented
  4. 4RED_FLAGS
    SBP <90 with lactate ≥2 → SCAI C+ — early MCS consideration given restrictive low-output state; AL with rapidly rising free light chains → oncologic emergency; ventricular arrhythmia common; AVOID typical inotropes (paradoxically worse)
    inputs: sbp, lactate, nt_probnp
    actions: cardiogenic_shock
    advance: Red flags screened + escalation pathway documented
  5. 5INITIAL_WORKUP
    NT-proBNP + troponin + BMP + lactate + ECG (low voltage, pseudo-infarct pattern) + echo with strain (apical sparing) + CXR + ABG; coags for AL bleeding diathesis (factor X deficiency)
    inputs: nt_probnp, troponin, echo_strain, ecg, lactate
    actions: acs_pathway, panel.cardiac, panel.renal, panel.coag, panel.abg
    advance: Baseline workup documented and ACS-mimic ruled out
  6. 6BRANCHING_WORKUP
    AL screen FIRST (SPEP + UPEP + immunofixation + serum free light chains) → if positive route to hematology emergent. If AL screen negative → TTR PYP scan; if PYP positive → ATTR diagnosed (no biopsy needed). If PYP negative + suspicion remains → endomyocardial biopsy with mass spectrometry typing. Then TTR genotyping (ATTRwt vs ATTRv) for family screening + transplant planning
    inputs: serum_free_light_chains, spep_upep_immunofixation, pyp_scan_or_emb
    advance: Amyloid type definitively assigned (AL vs ATTRwt vs ATTRv)
  7. 7DIFFERENTIAL
    AL vs ATTRwt vs ATTRv; consider mimics (HOCM, hypertensive heart, Fabry, sarcoidosis, hemochromatosis); rule out concurrent ACS (pseudo-STEMI ECG mimic)
    inputs: pyp_scan_or_emb
    advance: Amyloid type assigned and mimics excluded
  8. 8RISK_STRATIFICATION
    Mayo AL stage (NT-proBNP, troponin, dFLC); ATTR NAC stage (NT-proBNP + eGFR); SCAI 2022 CS stage; transplant eligibility — drives MCS bridge urgency vs comfort-focused care
    inputs: nt_probnp, troponin, creatinine, sbp, lactate
    advance: Stage documented and disposition trajectory chosen
  9. 9TREATMENT
    AL → Dara-CyBorD (daratumumab + bortezomib + cyclophosphamide + dexamethasone) per ANDROMEDA PMID 34077641 ± HSCT — hematology owns. ATTR → tafamidis 61 mg PO daily lifelong (ATTR-ACT PMID 30145930) ± gene-silencer (patisiran/vutrisiran/inotersen). CS support: NOREPINEPHRINE first-line; IABP for afterload reduction; VA-ECMO / Impella 5.5 bridge to therapy onset (months) or transplant. AVOID: digoxin (binds fibrils), BB (autonomic intolerance), non-DHP CCB (binds fibrils + AV block), large-volume diuresis, ACEi/ARB (orthostasis); CAUTIOUS gentle loop diuretic if congestion
    inputs: sbp, creatinine, lactate
    actions: protocol.cardiogenic_shock
    advance: Disease-modifying + CS support plan started + MCS decision documented
  10. 10DISPOSITION
    CICU for all amyloid CS; AL with cardiac stage III-IV → hematology + cards-onc co-management; advanced HF / transplant team consult for MCS bridge eligibility evaluation
    advance: Unit + multi-disciplinary team assigned + MCS / transplant pathway documented
  11. 11MONITORING
    Continuous hemodynamics, daily weight (gentle diuresis), BMP, lactate clearance; AL: monitor FLC weekly during chemotherapy, LFT for daratumumab; ATTR: NT-proBNP + 6MWD + echo at 6 mo on tafamidis (response usually 12-30 mo)
    inputs: creatinine, nt_probnp, lactate
    actions: panel.cardiac, panel.renal
    advance: Monitoring cadence set + therapy response trajectory documented
  12. 12FOLLOWUP
    Cardiology amyloid center quarterly; advanced HF / transplant team monthly if MCS-dependent; hematology q2-4w during AL chemo; ID surveillance during AL therapy (PJP prophylaxis); family ATTRv screening; cardiac rehab if MCS recovery achieved
    advance: Long-term follow-up booked + amyloid-center handoff complete + family screening initiated