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cardio.acute-hf.amyloidosis.v1

ADHF in cardiac amyloidosis (ATTR-CM and AL)

cardiologyacuteadultacuteinpatienttransitionoutpatient
Start encounter →Print handoutPRODUCTION

Phase E adjacent-disease variant of cardio.acute-hf.core.v1 — narrowed to ADHF in cardiac amyloidosis (AL light-chain and ATTR transthyretin). Phenotype-first triage drives entirely different treatment paths (oncologic CyBorD/daratumumab for AL vs disease-stabilizing tafamidis ± gene silencer for ATTR). Diagnostic algorithm: AL screen FIRST (SPEP + UPEP + serum free light chains) before TTR PYP scan. PYP positive (visual grade 2–3 + H/CL ≥1.5) with negative monoclonal screen = ATTR diagnosed without biopsy. EMB with mass spec for indeterminate cases. Critical AVOID list: digoxin contraindicated (binds amyloid fibrils); CCB-non-DHP avoided; ACEi/ARB/BB caution (autonomic + restrictive); diuretics with extreme caution (preload-dependent narrow window). Manifest pointer reuses cardio.acute-hf.core.v1 manifest. Design-brief reuses parent. Status INTEGRATED; authored 2026-05-14 by shard-06-cardio-acute (Phase E wave 7 adjacent-disease).

Entry points (5)

  • symptom
    HF symptoms + LV wall thickness ≥12 mm + no long-standing HTN → amyloidosis screen
    hf_with_thick_lv_walls_no_htn
  • symptom
    HF + low-voltage QRS on ECG + thick LV walls (voltage-mass mismatch — amyloid signature)
    hf_with_low_voltage_ecg
  • history
    Elderly male with bilateral carpal tunnel release history + new HF (ATTRwt clue)
    older_male_with_carpal_tunnel_history
  • lab_abnormality
    Monoclonal protein on SPEP/SFLC + HF symptoms → AL amyloidosis emergent workup
    monoclonal_protein_with_hf
  • imaging
    Apical sparing pattern on speckle-tracking strain echo (cherry-on-top) — amyloid signature
    apical_sparing_on_strain_echo

Required inputs (12)

  • 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
  • sbprequired
    vital • used at RED_FLAGS
    Hypotension highly common in amyloid (autonomic + restrictive); narrow tolerance for diuretics + vasodilators
  • nt_probnprequired
    lab • used at INITIAL_WORKUP
    Markedly elevated in amyloid (often >3000 even with normal LVEF); also a Mayo AL staging variable (>1800)
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Persistently elevated in amyloid (myocardial infiltration); Mayo AL stage variable (cTnT >0.025 or hsTnT >40)
  • serum_free_light_chainsrequired
    lab • used at BRANCHING_WORKUP
    MANDATORY before PYP read — rule out AL amyloidosis; 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; tafamidis dose unaffected; patisiran requires baseline LFT/eGFR
  • echo_strainrequired
    imaging • used at INITIAL_WORKUP
    Apical sparing pattern (preserved apical longitudinal strain with reduced basal strain) — amyloid signature; LV wall thickness + restrictive filling
  • 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)

12-phase flow (11)

  1. 1FRAME
    Cardiac amyloidosis presenting as ADHF; phenotype-first triage (AL = oncologic emergency vs ATTR = disease-modifying therapy)
    inputs: age
    advance: amyloid clinically suspected
  2. 2ENTRY
    HF + thick walls / low voltage / older with carpal tunnel / monoclonal protein → screen
    inputs: age
    advance: one entry trigger present
  3. 3CONTEXT
    Demographics, family history, neuropathy, prior monoclonal workup, baseline renal/hepatic function (KDIGO 2021)
    inputs: age, sbp, creatinine
    advance: context complete
  4. 4RED_FLAGS
    Hypotension precipitated by ACEi/ARB/BB withdrawal; cardiogenic shock; AL amyloidosis with rapidly rising free light chains (FLC); ventricular arrhythmia
    inputs: sbp, troponin, nt_probnp
    actions: cardiogenic_shock
    advance: red flags screened or escalated
  5. 5INITIAL_WORKUP
    NT-proBNP + troponin + BMP + ECG (low voltage, pseudo-infarct pattern) + echo with strain (apical sparing) + CXR
    inputs: nt_probnp, troponin, echo_strain
    actions: acute_pulm_edema, panel.cardiac, panel.renal
    advance: baseline workup documented
  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
    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)
    inputs: pyp_scan_or_emb
    advance: amyloid type assigned + mimics excluded
  8. 8RISK_STRATIFICATION
    Mayo AL stage (NT-proBNP, troponin, dFLC); ATTR NAC stage (NT-proBNP + eGFR); NYHA class — drives tafamidis benefit estimation (NYHA III may have less benefit)
    inputs: nt_probnp, troponin, creatinine
    advance: stage documented
  9. 9TREATMENT
    AL → CyBorD or daratumumab + dexamethasone (ANDROMEDA PMID 34077641) ± HSCT — hematology owns. ATTR → tafamidis 61 mg PO daily lifelong (ATTR-ACT PMID 30145930) ± gene-silencer (patisiran/vutrisiran PMID 30144829, 35262029). Supportive: gentle loop diuretic, midodrine for orthostasis, AVOID ACEi/ARB/BB/digoxin/CCB
    inputs: sbp, creatinine
    actions: protocol.cardiogenic_shock
    advance: disease-modifying + supportive plan started
  10. 10DISPOSITION
    Floor vs ICU; AL with cardiac stage III–IV → ICU + hematology
    advance: unit + multidisciplinary team assigned
  11. 11MONITORING
    Daily weight, BMP, gentle diuresis; AL: monitor FLC weekly during chemotherapy; ATTR: NT-proBNP + 6MWD + echo at 6 mo on tafamidis
    inputs: creatinine, nt_probnp
    actions: panel.renal
    advance: monitoring plan documented