Clinical Commander

All dossiers
cardio.al-amyloidosis-cardiac.chronic.v1

AL (light-chain) cardiac amyloidosis — chronic (cardiology arm)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

AL cardiac amyloidosis chronic — cardiology arm: AL-vs-ATTR pivot (tissue typing mandatory; PYP can be falsely positive in AL), near-oncologic urgency, hematology clone-directed (Dara-VCd ANDROMEDA ± ASCT) + cardiac-supportive amyloid-specific HF (no BB/ACEi/ARNi; CCB/digoxin toxic). Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (clone-referral + supportive axis + workups + calculators + panels), test_files, 5-PMID evidence object (high-confidence AL-specific citations only — no fabricated identifiers), chronic phases all present. INTEGRATED (not PRODUCTION): clone-directed regimen + ASCT/transplant marked non_pharm (hematology-owned); diuretic/MRA/midodrine/DOAC RxCUIs reused from validated cardio dossiers; SNOMED deferred. 9 trigger/special-pop branches: no-monoclonal→ATTR, Mayo IIIb, acquired factor-X bleeding, renal AL, autonomic neuropathy, AF/thrombus, transplant-eligible, inappropriate-GDMT, CKD.

Entry points (5)

  • lab_abnormality
    Abnormal serum FLC ratio / monoclonal on serum or urine immunofixation
    monoclonal_protein
  • imaging
    Echo: apical-sparing strain + thick walls + low ECG voltage
    apical_sparing_low_voltage
  • lab_abnormality
    Disproportionately high NT-proBNP / troponin with preserved EF
    markedly_elevated_nt_probnp
  • symptom
    HF with nephrotic-range proteinuria / orthostatic autonomic features
    hf_with_nephrotic_or_autonomic
  • history
    Known MGUS / myeloma / plasma-cell dyscrasia with new cardiac signs
    known_plasma_cell_dyscrasia

Required inputs (12)

  • agerequired
    demographic • used at RISK_STRATIFICATION
    Transplant/ASCT eligibility + chemo tolerability
  • serum_free_light_chainsrequired
    lab • used at INITIAL_WORKUP
    Abnormal κ/λ ratio + dFLC — diagnosis + Mayo staging + response
  • serum_urine_immunofixationrequired
    lab • used at INITIAL_WORKUP
    Monoclonal detection — defines AL vs ATTR pivot
  • nt_probnprequired
    lab • used at RISK_STRATIFICATION
    Mayo/European stage + organ-response monitoring
  • troponinrequired
    lab • used at RISK_STRATIFICATION
    Mayo stage component; cardiac AL prognostic
  • tissue_biopsy_typingrequired
    imaging • used at BRANCHING_WORKUP
    Congo-red + mass-spec typing confirms AL (not ATTR) — essential before clone-directed therapy
  • cardiac_phenotyperequired
    imaging • used at INITIAL_WORKUP
    Echo strain / CMR ECV-LGE — infiltrative cardiac involvement
  • renal_panel
    lab • used at CONTEXT
    Concurrent renal AL (nephrotic/dialysis) changes management
  • coag_factor_x
    lab • used at RISK_STRATIFICATION
    Acquired factor-X deficiency in AL — periprocedural bleeding risk
  • atrial_fibrillation
    history • used at CONTEXT
    AF — anticoagulate (intracardiac-thrombus risk), balance bleeding
  • current_medsrequired
    medication • used at TREATMENT
    Deprescribe BB/ACEi/ARNi; flag CCB/digoxin (toxic in amyloid)
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Functional status + chemo intensity decision

12-phase flow (12)

  1. 1FRAME
    AL cardiac amyloid suspected — near-oncologic urgency; AL vs ATTR pivot
    inputs: monoclonal_protein
    advance: AL suspected, ATForward pivot framed
  2. 2ENTRY
    Monoclonal + apical sparing + high NPs + nephrotic/autonomic + known dyscrasia
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Clone, organ involvement (renal/autonomic/GI/soft tissue), bleeding risk
    inputs: renal_panel, atrial_fibrillation
    advance: systemic organ + bleeding context complete
  4. 4RED_FLAGS
    Mayo stage IIIb, decompensation, acquired factor-X bleeding, malignant arrhythmia
    inputs: nt_probnp, troponin
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Serum FLC + serum/urine immunofixation, NT-proBNP/troponin, echo (apical sparing), ECG (low voltage)
    inputs: serum_free_light_chains, serum_urine_immunofixation, nt_probnp, cardiac_phenotype
    actions: panel.cardiac
    advance: monoclonal + cardiac phenotype documented
  6. 6BRANCHING_WORKUP
    Tissue biopsy + mass-spec typing (confirm AL, NOT ATTR), bone-marrow/clone work-up, CMR; if NO monoclonal → route ATTR engine (do not over-diagnose AL)
    inputs: tissue_biopsy_typing
    actions: preop_cardiac
    advance: amyloid typed as AL (or routed to ATTR)
  7. 7DIFFERENTIAL
    AL vs ATTR vs other amyloid vs HCM/HTN-LVH; MGUS-incidental vs causal clone
    inputs: serum_free_light_chains, tissue_biopsy_typing
    advance: AL with causal clone confirmed
  8. 8RISK_STRATIFICATION
    Mayo 2012 / European stage (NT-proBNP, troponin, dFLC); stage IIIb very poor prognosis
    inputs: nt_probnp, troponin, serum_free_light_chains, nyha_class
    advance: AL cardiac stage + chemo intensity assigned
  9. 9TREATMENT
    Hematology clone-directed (daratumumab-VCd ± ASCT — ANDROMEDA) + cardiac supportive (cautious diuretic ± MRA; AVOID BB/ACEi/ARNi; CCB/digoxin contraindicated; anticoagulate AF; treat autonomic OH)
    inputs: current_meds, nyha_class
    advance: clone-directed referral + cardiac supportive plan documented
  10. 10DISPOSITION
    Amyloidosis centre + hematology co-management; highly selected heart transplant → chemo/SCT sequence
    inputs: nyha_class
    actions: preop_cardiac
    advance: co-management + referral plan set
  11. 11MONITORING
    Serial NT-proBNP/troponin + dFLC (hematologic + cardiac organ response); arrhythmia/conduction surveillance
    inputs: nt_probnp, serum_free_light_chains
    actions: panel.cardiac
    advance: response-monitoring cadence documented
  12. 12FOLLOWUP
    Organ-response surveillance, relapse detection (rising dFLC), lifelong amyloid-centre care
    inputs: serum_free_light_chains
    advance: long-term + relapse plan documented