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cardio.attr-cardiomyopathy.chronic.v1

Transthyretin amyloid cardiomyopathy (ATTR-CM, ATTRv + ATTRwt)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

ATTR-CM chronic — non-biopsy diagnosis (PYP + AL exclusion first) + amyloid-specific HF (standard HFrEF GDMT does NOT apply; CCB/digoxin toxic); disease-modifying TTR stabilisers (tafamidis ATTR-ACT, acoramidis ATTRibute-CM) ± silencers (vutrisiran HELIOS-B, patisiran APOLLO-B, eplontersen). Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (disease-modifying + amyloid-specific HF axis + workups + calculators + panels), test_files, 10-PMID evidence object, chronic phases all present. Novel ATTR drug RxCUIs RxNav-validated 2026-05-16 (tafamidis 1545063, acoramidis 2698312, patisiran 2053490, vutrisiran 2604578, eplontersen 2671939 — forward + reverse name lookup confirmed); decongestion/MRA/SGLT2i/DOAC RxCUIs reused from validated cardio dossiers; devices/transplant/TAVR non_pharm. HELIOS-B/CARDIO-TTRansform referenced by trial name (PMID pending verification — evidence-gap, not fabricated). 9 trigger/special-pop branches: monoclonal→AL, inappropriate-GDMT deprescribe, ATTRv genetic, ATTR-AS overlap, AF anticoagulation, conduction disease, advanced NAC stage III, orthopedic prodrome, CKD.

Entry points (6)

  • imaging
    HFpEF with LV wall thickness ≥12 mm not explained by HTN/AS
    unexplained_lvh_hfpef
  • lab_abnormality
    Low ECG voltage discordant with increased LV wall thickness
    discordant_low_ecg_voltage
  • history
    Bilateral carpal tunnel syndrome / lumbar canal stenosis / biceps rupture (amyloid prodrome)
    bilateral_carpal_tunnel
  • imaging
    99mTc-PYP / DPD scintigraphy Perugini grade 2–3
    pyp_positive
  • imaging
    Echo: apical-sparing relative longitudinal strain pattern
    apical_sparing_strain
  • history
    Family history / known TTR variant (ATTRv)
    family_history_attrv

Required inputs (14)

  • agerequired
    demographic • used at CONTEXT
    ATTRwt typically >65; staging + drug eligibility
  • ancestry
    demographic • used at CONTEXT
    V122I (pV142I) common in people of African ancestry — directs TTR genotyping
  • serum_free_light_chainsrequired
    lab • used at INITIAL_WORKUP
    AL EXCLUSION FIRST — abnormal κ/λ ratio mandates AL pathway before PYP can diagnose ATTR
  • serum_urine_immunofixationrequired
    lab • used at INITIAL_WORKUP
    Monoclonal protein detection — must be negative for non-biopsy ATTR diagnosis
  • pyp_scintigraphy_graderequired
    imaging • used at BRANCHING_WORKUP
    Perugini grade 2–3 with negative monoclonal = ATTR-CM without biopsy
  • lv_wall_thicknessrequired
    imaging • used at INITIAL_WORKUP
    Infiltrative LVH; discordant with low voltage
  • nt_probnprequired
    lab • used at RISK_STRATIFICATION
    NAC/Mayo ATTR staging + monitoring
  • troponin
    lab • used at RISK_STRATIFICATION
    Mayo ATTR stage component
  • creatininerequired
    lab • used at RISK_STRATIFICATION
    NAC stage (eGFR) + drug considerations
  • ttr_genotype
    history • used at BRANCHING_WORKUP
    ATTRv vs ATTRwt — genetic counseling, cascade, polyneuropathy overlap
  • atrial_fibrillation
    history • used at CONTEXT
    AF — anticoagulate regardless of CHA₂DS₂-VASc (high intracardiac-thrombus risk)
  • aortic_stenosis
    history • used at CONTEXT
    ATTR-AS overlap — TAVR consideration
  • current_medsrequired
    medication • used at TREATMENT
    Detect/deprescribe BB, ACEi/ARNi, non-DHP CCB, digoxin (poorly tolerated/toxic in amyloid)
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    NYHA I–III is the drug-trial-eligible band for stabilisers

12-phase flow (12)

  1. 1FRAME
    Suspect ATTR-CM (HFpEF + infiltrative LVH + red flags); explicitly NOT standard HFrEF
    inputs: lv_wall_thickness
    advance: ATTR-CM clinically suspected
  2. 2ENTRY
    HFpEF + LVH, discordant low voltage, carpal-tunnel prodrome, PYP-positive, ATTRv family history
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Age/sex, ancestry, family history, neuropathy, orthopedic prodrome, AF, AS
    inputs: ancestry, atrial_fibrillation, aortic_stenosis
    advance: context + red-flag prodrome catalogued
  4. 4RED_FLAGS
    Decompensation, high-grade conduction block, intracardiac thrombus
    inputs: nyha_class
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo (apical sparing), ECG, NT-proBNP/troponin, and AL EXCLUSION FIRST (serum FLC + serum/urine immunofixation)
    inputs: serum_free_light_chains, serum_urine_immunofixation, lv_wall_thickness, nt_probnp
    actions: panel.cardiac
    advance: AL screen resulted; infiltrative phenotype documented
  6. 6BRANCHING_WORKUP
    If monoclonal NEGATIVE → 99mTc-PYP/DPD + CMR (ECV/LGE) + TTR genotyping; if monoclonal POSITIVE → AL amyloid pathway (biopsy/heme), do NOT diagnose ATTR by PYP alone
    inputs: pyp_scintigraphy_grade, ttr_genotype
    actions: preop_cardiac
    advance: ATTR confirmed (PYP 2–3 + monoclonal negative) or routed to AL
  7. 7DIFFERENTIAL
    ATTR-CM vs AL amyloid vs HCM vs hypertensive LVH vs Fabry vs HFpEF vs ATTR-AS overlap
    inputs: serum_free_light_chains, pyp_scintigraphy_grade
    advance: amyloid type + genotype assigned
  8. 8RISK_STRATIFICATION
    NAC stage (NT-proBNP + eGFR) / Mayo stage (NT-proBNP + troponin); genotype; NYHA
    inputs: nt_probnp, troponin, creatinine, nyha_class
    advance: ATTR stage + drug eligibility assigned
  9. 9TREATMENT
    TTR stabiliser (tafamidis/acoramidis) ± silencer (patisiran/vutrisiran/eplontersen); cautious loop-diuretic decongestion ± MRA; AVOID/deprescribe BB, ACEi/ARB/ARNi, non-DHP CCB, digoxin; anticoagulate AF regardless of CHA₂DS₂-VASc
    inputs: current_meds, nyha_class
    advance: disease-modifying + amyloid-specific HF plan documented
  10. 10DISPOSITION
    Amyloidosis centre referral; genetic counseling (ATTRv); transplant for selected young ATTRv
    inputs: ttr_genotype
    actions: preop_cardiac
    advance: specialist referral + counseling plan set
  11. 11MONITORING
    Serial NT-proBNP/troponin/echo/stage; device + conduction surveillance
    inputs: nt_probnp, creatinine
    actions: panel.cardiac, panel.renal
    advance: monitoring cadence documented
  12. 12FOLLOWUP
    Family cascade screening (ATTRv); ATTR-AS → TAVR; lifelong amyloid-centre care
    inputs: ttr_genotype
    advance: cascade + long-term plan documented