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cardio.al-amyloidosis-cardiac.chronic.v1PRODUCTION
cardio.al-amyloidosis-cardiac.chronic.v1

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

cardiologychronicadult
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Care setting:

Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

AL cardiac amyloid suspected — near-oncologic urgency; AL vs ATTR pivot

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AL suspected, ATForward pivot framed

Patient inputs (12)

Congo-red + mass-spec typing confirms AL (not ATTR) — essential before clone-directed therapy

Abnormal κ/λ ratio + dFLC — diagnosis + Mayo staging + response

Monoclonal detection — defines AL vs ATTR pivot

Echo strain / CMR ECV-LGE — infiltrative cardiac involvement

Transplant/ASCT eligibility + chemo tolerability

Mayo/European stage + organ-response monitoring

Mayo stage component; cardiac AL prognostic

Functional status + chemo intensity decision

Deprescribe BB/ACEi/ARNi; flag CCB/digoxin (toxic in amyloid)

Concurrent renal AL (nephrotic/dialysis) changes management

AF — anticoagulate (intracardiac-thrombus risk), balance bleeding

Acquired factor-X deficiency in AL — periprocedural bleeding risk

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (9)

9 need judgement
  • informationallife_threateningmayo_stage_iiib
    Mayo stage IIIb (very high NT-proBNP + troponin) — very poor prognosis; attenuated/adapted chemo, early palliative-care integration — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereacquired_factor_x_bleeding
    Acquired factor-X deficiency (amyloid adsorption) — periprocedural bleeding risk; factor support / hematology before invasive procedures — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererenal_al_branch
    Concurrent renal AL (nephrotic-range proteinuria / declining eGFR / dialysis) — diuretic-resistant edema, drug dosing, dual-organ staging — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaf_thrombus_branch
    AF or intracardiac thrombus in cardiac AL — anticoagulate regardless of CHA₂DS₂-VASc, balanced against factor-X bleeding — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveretransplant_eligible_branch
    Advanced cardiac AL otherwise transplant-eligible with controllable clone — heart transplant then clone-directed/SCT sequence at selected centres — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinappropriate_gdmt_in_al
    On beta-blocker / ACEi-ARB-ARNi / non-DHP CCB / digoxin — deprescribe (amyloid-specific HF; CCB/digoxin toxic) — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateno_monoclonal_route_attr
    No monoclonal protein on FLC + immunofixation — this is NOT AL; route to ATTR engine (PYP-based) — Gillmore Circulation 2016
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateautonomic_neuropathy_branch
    Autonomic neuropathy (severe orthostatic hypotension, GI dysmotility) — limits diuretic/vasoactive titration; midodrine + compression + salt — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD (renal AL or otherwise) — dose-gate supportive drugs; staging interaction — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

AL cardiac — clone-directed referral + cardiac-supportive amyloid-specific HF (ANDROMEDA; 2023 ACC amyloid ECDP)
axis: al_cardiac_clone_directed_referral_plus_supportivestep 1 - Step 1 — Confirm AL (typed) + stage; route ATTR-out if no monoclonal
Selected step "Step 1 — Confirm AL (typed) + stage; route ATTR-out if no monoclonal" — Monoclonal protein present + amyloid on tissue typed as AL

outpatient playbook — drug actions (3)

  1. 1. urgent hematology referral for Dara-VCd
    hematology-managed • IV/SC/PO • per protocol
    trigger: Confirmed cardiac AL (ANDROMEDA)
    Clone-directed therapy is the only disease-modifying treatment
  2. 2. cautious loop diuretic ± MRA
    furosemide 20–40 mg • PO • daily
    trigger: Congestion (2023 ACC amyloid ECDP)
    Supportive — not 4-pillar GDMT
  3. 3. midodrine for autonomic OH; apixaban if AF
    midodrine 2.5–5 mg TID • PO • TID/BID
    trigger: Autonomic OH / AF (2023 ACC amyloid ECDP)
    Symptom + thrombo-prophylaxis management

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Abnormal serum FLC ratio / monoclonal on serum or urine immunofixation; Echo: apical-sparing strain + thick walls + low ECG voltage; Disproportionately high NT-proBNP / troponin with preserved EF.

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**AL (light-chain) cardiac amyloidosis — chronic (cardiology arm)** (cardio.al-amyloidosis-cardiac.chronic.v1).
Phenotype framing: AL vs ATTR vs other amyloid vs HCM/HTN-LVH; MGUS-incidental vs causal clone
Scope: AL cardiac amyloid suspected — near-oncologic urgency; AL vs ATTR pivot

No severity triggers fired against current inputs.

Plan

Regimen axis: **AL cardiac — clone-directed referral + cardiac-supportive amyloid-specific HF (ANDROMEDA; 2023 ACC amyloid ECDP)** — step "Step 1 — Confirm AL (typed) + stage; route ATTR-out if no monoclonal".

Setting playbook (outpatient) — Confirm/type AL, stage, refer hematology urgently for clone-directed therapy, co-manage cardiac supportive care (ANDROMEDA; 2023 ACC amyloid ECDP)
1. urgent hematology referral for Dara-VCd hematology-managed IV/SC/PO per protocol — Confirmed cardiac AL (ANDROMEDA) (Clone-directed therapy is the only disease-modifying treatment)
2. cautious loop diuretic ± MRA furosemide 20–40 mg PO daily — Congestion (2023 ACC amyloid ECDP) (Supportive — not 4-pillar GDMT)
3. midodrine for autonomic OH; apixaban if AF midodrine 2.5–5 mg TID PO TID/BID — Autonomic OH / AF (2023 ACC amyloid ECDP) (Symptom + thrombo-prophylaxis management)

Non-pharmacologic actions:
- Urgent amyloidosis-centre + hematology co-management — ANDROMEDA
- Deprescribe beta-blockers/ACEi/ARB/ARNi/CCB/digoxin — 2023 ACC amyloid ECDP
- Compression garments + salt/fluid for autonomic OH — 2023 ACC amyloid ECDP

AVOID / contraindication checks:
- AL is near oncologic urgency refer hematology immediately — ANDROMEDA
- Standard HFrEF GDMT does not apply in cardiac AL — 2023 ACC amyloid ECDP
- Avoid beta blockers ACEi ARB ARNi hypotension autonomic — 2023 ACC amyloid ECDP
- Non DHP CCB and digoxin CONTRAINDICATED amyloid binding — 2023 ACC amyloid ECDP
- Assess acquired factor X before invasive procedures — 2023 ACC amyloid ECDP
- Do not diagnose AL without tissue typing PYP can be falsely positive — Gillmore Circulation 2016

Monitoring

Regimen monitoring:
- dFLC and hematologic response per hematology — ANDROMEDA
- NT-proBNP troponin for cardiac organ response q1-3mo — 2023 ACC amyloid ECDP
- echo strain and conduction surveillance — 2023 ACC amyloid ECDP
- orthostatic BP for autonomic dysfunction — 2023 ACC amyloid ECDP
- factor X and bleeding assessment periprocedurally — 2023 ACC amyloid ECDP

Setting (outpatient) monitoring:
- dFLC + hematologic response (hematology); NT-proBNP/troponin cardiac response — ANDROMEDA; 2023 ACC amyloid ECDP
- Orthostatic BP + conduction surveillance — 2023 ACC amyloid ECDP

Follow-up plan: Organ-response surveillance, relapse detection (rising dFLC), lifelong amyloid-centre care
- Close-out criterion: long-term + relapse plan documented

Monitoring phase: Serial NT-proBNP/troponin + dFLC (hematologic + cardiac organ response); arrhythmia/conduction surveillance

Disposition

Current setting: outpatient — Confirm/type AL, stage, refer hematology urgently for clone-directed therapy, co-manage cardiac supportive care (ANDROMEDA; 2023 ACC amyloid ECDP)

Disposition criteria:
- Confirmed AL → urgent hematology + cardiac co-management
- Transplant-eligible advanced cardiac AL → transplant-then-clone sequence (select centres)
- No monoclonal → ATTR pathway

Escalation triggers (move to higher acuity):
- Mayo stage IIIb / rapid decompensation → palliative-leaning + attenuated chemo discussion — 2023 ACC amyloid ECDP
- Acquired factor-X bleeding before procedure → hematology / factor support — 2023 ACC amyloid ECDP
- No monoclonal on work-up → route ATTR engine (do not treat as AL) — Gillmore Circulation 2016

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Mayo stage IIIb (very high NT-proBNP + troponin) — very poor prognosis; attenuated/adapted chemo, early palliative-care integration — 2023 ACC amyloid ECDP
- [SEVERE] Acquired factor-X deficiency (amyloid adsorption) — periprocedural bleeding risk; factor support / hematology before invasive procedures — 2023 ACC amyloid ECDP
- [SEVERE] Concurrent renal AL (nephrotic-range proteinuria / declining eGFR / dialysis) — diuretic-resistant edema, drug dosing, dual-organ staging — 2023 ACC amyloid ECDP

Citations

- 2023 ACC Cardiac Amyloidosis Expert Consensus Decision Pathway + ANDROMEDA (Dara-VCd) + revised Mayo/European staging [PMID:34192431](https://pubmed.ncbi.nlm.nih.gov/34192431/)
- Cited evidence (PMID 22331955) [PMID:22331955](https://pubmed.ncbi.nlm.nih.gov/22331955/)
- Cited evidence (PMID 27143678) [PMID:27143678](https://pubmed.ncbi.nlm.nih.gov/27143678/)
- Cited evidence (PMID 35379504) [PMID:35379504](https://pubmed.ncbi.nlm.nih.gov/35379504/)
- Cited evidence (PMID 37622666) [PMID:37622666](https://pubmed.ncbi.nlm.nih.gov/37622666/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2023 ACC Cardiac Amyloidosis Expert Consensus Decision Pathway + ANDROMEDA (Dara-VCd) + revised Mayo/European staging — PMID:34192431
  • Cited evidence (PMID 22331955) — PMID:22331955
  • Cited evidence (PMID 27143678) — PMID:27143678
  • Cited evidence (PMID 35379504) — PMID:35379504
  • Cited evidence (PMID 37622666) — PMID:37622666