Clinical Commander

Back to dossier
cardio.attr-cardiomyopathy.chronic.v1PRODUCTION
cardio.attr-cardiomyopathy.chronic.v1

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

cardiologychronicadult
Hard-required inputs
0 / 9
Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Suspect ATTR-CM (HFpEF + infiltrative LVH + red flags); explicitly NOT standard HFrEF

Inputs
1
Actions
0
Advance rule
Set
Advance when

ATTR-CM clinically suspected

Patient inputs (14)

Perugini grade 2–3 with negative monoclonal = ATTR-CM without biopsy

ATTRwt typically >65; staging + drug eligibility

AL EXCLUSION FIRST — abnormal κ/λ ratio mandates AL pathway before PYP can diagnose ATTR

Monoclonal protein detection — must be negative for non-biopsy ATTR diagnosis

Infiltrative LVH; discordant with low voltage

NAC/Mayo ATTR staging + monitoring

NAC stage (eGFR) + drug considerations

NYHA I–III is the drug-trial-eligible band for stabilisers

Detect/deprescribe BB, ACEi/ARNi, non-DHP CCB, digoxin (poorly tolerated/toxic in amyloid)

ATTRv vs ATTRwt — genetic counseling, cascade, polyneuropathy overlap

V122I (pV142I) common in people of African ancestry — directs TTR genotyping

AF — anticoagulate regardless of CHA₂DS₂-VASc (high intracardiac-thrombus risk)

ATTR-AS overlap — TAVR consideration

Mayo ATTR stage component

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

Severity triggers (9)

9 need judgement
  • informationalseveremonoclonal_positive_route_AL
    Abnormal serum FLC ratio or monoclonal on immunofixation — do NOT diagnose ATTR by PYP; route to AL amyloid / hematology pathway (oncologic) — Gillmore Circulation 2016
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereinappropriate_gdmt_in_attr
    Patient on beta-blocker / ACEi-ARB-ARNi / non-DHP CCB / digoxin — deprescribe; standard HFrEF GDMT does not apply and CCB/digoxin are toxic in amyloid — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereattr_as_overlap_branch
    Concomitant severe aortic stenosis + ATTR-CM (ATTR-AS) — TAVR consideration; do not deny intervention for amyloid alone — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaf_in_attr_anticoagulate
    AF in ATTR-CM — anticoagulate regardless of CHA₂DS₂-VASc (high intracardiac-thrombus/stroke risk); rate control difficult (avoid BB/non-DHP CCB/digoxin) — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereconduction_disease_branch
    High-grade AV block / progressive conduction disease (amyloid infiltration of conduction system) — pacemaker — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereadvanced_nac_stage_iii
    NAC stage III (high NT-proBNP + low eGFR) — TTR-stabiliser benefit attenuated; emphasise symptom control, transplant assessment (young ATTRv), palliative discussion — ATTR-ACT subgroup
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateattrv_genetic_branch
    ATTRv genotype (V122I/T60A/V30M etc.) — genetic counseling + first-degree family cascade screening; polyneuropathy overlap → neuro — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — NAC staging uses eGFR; consider renal amyloid; drug/decongestion adjustment — KDIGO 2024; 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildorthopedic_prodrome_early_detection
    Bilateral carpal tunnel / lumbar canal stenosis / spontaneous biceps tendon rupture — amyloid prodrome preceding cardiomyopathy by years; low threshold to screen — 2023 ACC amyloid ECDP
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONrequiredDrives risk stratification
Loading…

Recommended regimen

ATTR-CM disease-modifying + amyloid-specific HF (ATTR-ACT/ATTRibute-CM/APOLLO-B/HELIOS-B; 2023 ACC amyloid ECDP)
axis: attr_cm_disease_modifying_and_amyloid_specific_hfstep 1 - Step 1 — Confirm ATTR (AL excluded) + stage; route AL out if monoclonal positive
Selected step "Step 1 — Confirm ATTR (AL excluded) + stage; route AL out if monoclonal positive" — PYP grade 2–3 AND serum FLC + serum/urine immunofixation NEGATIVE

outpatient playbook — drug actions (4)

  1. 1. tafamidis or acoramidis
    tafamidis 61 mg daily / acoramidis 800 mg BID • PO • daily/BID
    trigger: Confirmed ATTR-CM NYHA I–III (ATTR-ACT/ATTRibute-CM)
    Mortality + CV-hospitalisation benefit
  2. 2. vutrisiran (add/alternative; ATTRv or progression)
    25 mg SC q3 mo • SC • q3 months
    trigger: ATTR-CM progression / ATTRv polyneuropathy (HELIOS-B)
    TTR knockdown — CV-event reduction
  3. 3. loop diuretic ± MRA ± SGLT2i (amyloid-specific HF)
    furosemide 20–40 mg • PO • daily
    trigger: Congestion (2023 ACC amyloid ECDP)
    Mainstay decongestion — NOT 4-pillar GDMT
  4. 4. anticoagulation if AF
    apixaban 5 mg BID • PO • BID
    trigger: Any AF in ATTR-CM (2023 ACC amyloid ECDP)
    Anticoagulate regardless of CHA₂DS₂-VASc

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: HFpEF with LV wall thickness ≥12 mm not explained by HTN/AS; Low ECG voltage discordant with increased LV wall thickness; Bilateral carpal tunnel syndrome / lumbar canal stenosis / biceps rupture (amyloid prodrome).

Objective

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

Assessment

**Transthyretin amyloid cardiomyopathy (ATTR-CM, ATTRv + ATTRwt)** (cardio.attr-cardiomyopathy.chronic.v1).
Phenotype framing: ATTR-CM vs AL amyloid vs HCM vs hypertensive LVH vs Fabry vs HFpEF vs ATTR-AS overlap
Scope: Suspect ATTR-CM (HFpEF + infiltrative LVH + red flags); explicitly NOT standard HFrEF

No severity triggers fired against current inputs.

Plan

Regimen axis: **ATTR-CM disease-modifying + amyloid-specific HF (ATTR-ACT/ATTRibute-CM/APOLLO-B/HELIOS-B; 2023 ACC amyloid ECDP)** — step "Step 1 — Confirm ATTR (AL excluded) + stage; route AL out if monoclonal positive".

Setting playbook (outpatient) — Confirm ATTR (AL excluded) by non-biopsy algorithm, stage, start TTR stabiliser/silencer, apply amyloid-specific HF rules, cascade-screen ATTRv (2023 ACC amyloid ECDP)
1. tafamidis or acoramidis tafamidis 61 mg daily / acoramidis 800 mg BID PO daily/BID — Confirmed ATTR-CM NYHA I–III (ATTR-ACT/ATTRibute-CM) (Mortality + CV-hospitalisation benefit)
2. vutrisiran (add/alternative; ATTRv or progression) 25 mg SC q3 mo SC q3 months — ATTR-CM progression / ATTRv polyneuropathy (HELIOS-B) (TTR knockdown — CV-event reduction)
3. loop diuretic ± MRA ± SGLT2i (amyloid-specific HF) furosemide 20–40 mg PO daily — Congestion (2023 ACC amyloid ECDP) (Mainstay decongestion — NOT 4-pillar GDMT)
4. anticoagulation if AF apixaban 5 mg BID PO BID — Any AF in ATTR-CM (2023 ACC amyloid ECDP) (Anticoagulate regardless of CHA₂DS₂-VASc)

Non-pharmacologic actions:
- Deprescribe beta-blockers, ACEi/ARB/ARNi, non-DHP CCB, digoxin — 2023 ACC amyloid ECDP
- Genetic counseling + first-degree family cascade screening for ATTRv — 2023 ACC amyloid ECDP
- Amyloidosis-centre referral; transplant evaluation in selected young ATTRv — 2023 ACC amyloid ECDP
- TAVR evaluation if concomitant severe AS (ATTR-AS) — 2023 ACC amyloid ECDP

AVOID / contraindication checks:
- Exclude AL with FLC and immunofixation before diagnosing ATTR by PYP — Gillmore Circulation 2016
- Standard HFrEF GDMT does not apply in ATTR CM — 2023 ACC amyloid ECDP
- Avoid or deprescribe beta blockers poorly tolerated fixed stroke volume — 2023 ACC amyloid ECDP
- Avoid ACEi ARB ARNi hypotension autonomic neuropathy — 2023 ACC amyloid ECDP
- Non DHP CCB and digoxin CONTRAINDICATED bind amyloid fibrils toxicity — 2023 ACC amyloid ECDP
- Anticoagulate AF in ATTR CM regardless of CHA2DS2VASc — 2023 ACC amyloid ECDP
- Stabiliser benefit attenuates in advanced NAC stage III — ATTR ACT subgroup

Monitoring

Regimen monitoring:
- NT-proBNP troponin eGFR for NAC Mayo stage q6-12mo — 2023 ACC amyloid ECDP
- echo with strain annually — 2023 ACC amyloid ECDP
- ECG and device check for conduction disease — 2023 ACC amyloid ECDP
- TTR genotyping once then family cascade if ATTRv — 2023 ACC amyloid ECDP
- medication reconciliation to deprescribe BB ACEi CCB digoxin — 2023 ACC amyloid ECDP

Setting (outpatient) monitoring:
- NT-proBNP/troponin/eGFR + echo strain at 6–12 mo — 2023 ACC amyloid ECDP
- Conduction surveillance (ECG/device) — 2023 ACC amyloid ECDP

Follow-up plan: Family cascade screening (ATTRv); ATTR-AS → TAVR; lifelong amyloid-centre care
- Close-out criterion: cascade + long-term plan documented

Monitoring phase: Serial NT-proBNP/troponin/echo/stage; device + conduction surveillance

Disposition

Current setting: outpatient — Confirm ATTR (AL excluded) by non-biopsy algorithm, stage, start TTR stabiliser/silencer, apply amyloid-specific HF rules, cascade-screen ATTRv (2023 ACC amyloid ECDP)

Disposition criteria:
- Confirmed ATTR-CM NYHA I–III → start stabiliser + amyloid-centre follow-up
- Monoclonal positive → AL amyloid pathway (do not treat as ATTR)
- Advanced NAC stage III → palliative-leaning + transplant assessment (young ATTRv)

Escalation triggers (move to higher acuity):
- Decompensation → cautious decongestion, amyloid-acute pathway — 2023 ACC amyloid ECDP
- High-grade AV block → pacemaker — 2023 ACC amyloid ECDP
- Monoclonal protein detected → AL pathway / hematology — Gillmore Circulation 2016

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] Abnormal serum FLC ratio or monoclonal on immunofixation — do NOT diagnose ATTR by PYP; route to AL amyloid / hematology pathway (oncologic) — Gillmore Circulation 2016
- [SEVERE] Patient on beta-blocker / ACEi-ARB-ARNi / non-DHP CCB / digoxin — deprescribe; standard HFrEF GDMT does not apply and CCB/digoxin are toxic in amyloid — 2023 ACC amyloid ECDP
- [SEVERE] Concomitant severe aortic stenosis + ATTR-CM (ATTR-AS) — TAVR consideration; do not deny intervention for amyloid alone — 2023 ACC amyloid ECDP

Citations

- 2023 ACC Cardiac Amyloidosis Expert Consensus Decision Pathway + 2022 AHA/ACC/HFSA HF Guideline; Gillmore non-biopsy diagnostic algorithm [PMID:30145929](https://pubmed.ncbi.nlm.nih.gov/30145929/)
- Cited evidence (PMID 38122933) [PMID:38122933](https://pubmed.ncbi.nlm.nih.gov/38122933/)
- Cited evidence (PMID 37888914) [PMID:37888914](https://pubmed.ncbi.nlm.nih.gov/37888914/)
- 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/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2023 ACC Cardiac Amyloidosis Expert Consensus Decision Pathway + 2022 AHA/ACC/HFSA HF Guideline; Gillmore non-biopsy diagnostic algorithm — PMID:30145929
  • Cited evidence (PMID 38122933) — PMID:38122933
  • Cited evidence (PMID 37888914) — PMID:37888914
  • Cited evidence (PMID 27143678) — PMID:27143678
  • Cited evidence (PMID 35379504) — PMID:35379504