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cardio.restrictive-cardiomyopathy.chronic.v1PRODUCTION
cardio.restrictive-cardiomyopathy.chronic.v1

Restrictive cardiomyopathy (chronic — etiology hunt + RCM-vs-CP pivot)

cardiologychronicadult
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12/12 authored

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

Current phase

Frame

Detailed

Confirm restrictive physiology; immediately set up the RCM-vs-constrictive-pericarditis question (CP is curable)

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Advance rule
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Advance when

restrictive physiology confirmed; CP question framed

Patient inputs (12)

Invasive hemodynamics (ventricular interdependence, respiratory discordance) — RCM vs constrictive pericarditis pivot

Amyloid screen (route AL/ATTR) — most common infiltrative RCM

Pediatric familial vs adult acquired; prognosis

Restrictive Doppler/strain + biatrial enlargement defines the phenotype

Typically higher in RCM than constrictive pericarditis; prognosis

Functional status + transplant timing

Diuretic + drug dosing; cardiorenal in restrictive physiology

CMR LGE pattern + pericardium — etiology + RCM-vs-CP

Iron-overload/hemochromatosis screen (T2* MRI if positive)

Fabry screen (males); GLA gene

Loeffler/hypereosinophilic endomyocardial disease

AF with biatrial enlargement — high thrombus risk → anticoagulate

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

Severity triggers (9)

9 need judgement
  • informationalsevereconstrictive_pericarditis_pivot
    Hemodynamics/imaging indicate constrictive pericarditis rather than RCM — pericardiectomy is potentially CURATIVE; do not mislabel RCM — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereamyloid_etiology_branch
    Amyloid as the RCM etiology (FLC/PYP) — route to ATTR or AL engine for disease-modifying therapy — Gillmore Circulation 2016
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverefabry_branch
    Fabry disease (low α-Gal in males / GLA variant) — enzyme replacement / migalastat; route metabolic pathway — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereiron_overload_branch
    Iron-overload cardiomyopathy (high ferritin/TSAT, low cardiac T2*) — phlebotomy/chelation; route iron-overload pathway — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereloeffler_hes_branch
    Loeffler endomyocardial disease / hypereosinophilic syndrome — corticosteroids ± imatinib (FIP1L1-PDGFRA); anticoagulate for endomyocardial thrombus — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereidiopathic_familial_branch
    Idiopathic/familial (sarcomeric/desmin/TNNI3) RCM — poor prognosis; genetics + transplant evaluation — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereaf_atrial_thrombus_branch
    AF with marked biatrial enlargement — high intracardiac-thrombus/embolic risk → anticoagulate; gentle rate control preserving filling time — ESC 2024 AF
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with RCM — restrictive physiology poorly tolerates volume shifts; high maternal risk; cardio-obstetric, careful diuresis, avoid ACEi/ARB — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD/cardiorenal — diuretic resistance management, drug dose-gating — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

RCM etiology routing + cautious symptomatic HF (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)
axis: rcm_etiology_routing_and_symptomaticstep 1 - Step 1 — Resolve RCM vs constrictive pericarditis (CP is curable)
Selected step "Step 1 — Resolve RCM vs constrictive pericarditis (CP is curable)" — Restrictive physiology established
  • pericardiectomy referral (if constrictive pericarditis)
    rescue
    procedure
    triggers: constrictive_pericarditis_confirmed
    CP is potentially curable by pericardiectomy — must not be mislabeled RCM (2023 ESC Cardiomyopathy)

outpatient playbook — drug actions (3)

  1. 1. pericardiectomy referral if CP
    n/a • surgical • n/a
    trigger: Constrictive pericarditis confirmed (2023 ESC Cardiomyopathy)
    Potentially curative
  2. 2. route etiology-specific therapy
    per etiology • varied • varied
    trigger: Identified RCM etiology
    Disease-modifying therapy is etiology-specific
  3. 3. cautious loop diuretic ± MRA; anticoagulate AF
    furosemide 20–40 mg; apixaban if AF • PO • daily/BID
    trigger: Congestion / AF (2023 ESC Cardiomyopathy)
    Symptomatic + thromboprophylaxis

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Echo: restrictive filling + biatrial enlargement + non-dilated ventricles + normal EF; Right-heart congestion (edema, ascites, elevated JVP) with preserved EF; Exertional dyspnea / fatigue out of proportion to EF.

Objective

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

Assessment

**Restrictive cardiomyopathy (chronic — etiology hunt + RCM-vs-CP pivot)** (cardio.restrictive-cardiomyopathy.chronic.v1).
Phenotype framing: RCM etiologies (amyloid/Fabry/iron/Loeffler/idiopathic-familial) vs constrictive pericarditis
Scope: Confirm restrictive physiology; immediately set up the RCM-vs-constrictive-pericarditis question (CP is curable)

No severity triggers fired against current inputs.

Plan

Regimen axis: **RCM etiology routing + cautious symptomatic HF (2023 ESC Cardiomyopathy; 2022 AHA/ACC/HFSA HF)** — step "Step 1 — Resolve RCM vs constrictive pericarditis (CP is curable)".
1. pericardiectomy referral (if constrictive pericarditis) (procedure, rescue) — CP is potentially curable by pericardiectomy — must not be mislabeled RCM (2023 ESC Cardiomyopathy)

Setting playbook (outpatient) — Resolve RCM-vs-CP, hunt + route etiology, manage congestion cautiously, anticoagulate AF (2023 ESC Cardiomyopathy)
2. pericardiectomy referral if CP n/a surgical n/a — Constrictive pericarditis confirmed (2023 ESC Cardiomyopathy) (Potentially curative)
3. route etiology-specific therapy per etiology varied varied — Identified RCM etiology (Disease-modifying therapy is etiology-specific)
4. cautious loop diuretic ± MRA; anticoagulate AF furosemide 20–40 mg; apixaban if AF PO daily/BID — Congestion / AF (2023 ESC Cardiomyopathy) (Symptomatic + thromboprophylaxis)

Non-pharmacologic actions:
- Route to amyloid/Fabry/iron/HES/sarcoid engines for disease-modifying therapy — 2023 ESC Cardiomyopathy
- Transplant evaluation for end-stage idiopathic/familial RCM — 2023 ESC Cardiomyopathy
- Genetic counseling + family cascade if familial — 2023 ESC Cardiomyopathy

AVOID / contraindication checks:
- Exclude constrictive pericarditis it is curable by pericardiectomy — 2023 ESC Cardiomyopathy
- Standard HFrEF GDMT generally not applicable preserved EF restrictive — 2023 ESC Cardiomyopathy
- Avoid aggressive afterload reduction preload dependent stiff ventricle — 2023 ESC Cardiomyopathy
- Anticoagulate AF high atrial thrombus risk in biatrial enlargement — ESC 2024 AF
- Route amyloid etiology to ATTR or AL engine for disease modifying therapy — 2023 ESC Cardiomyopathy

Monitoring

Regimen monitoring:
- etiology specific monitoring per routed engine — 2023 ESC Cardiomyopathy
- serial echo atrial size and filling — 2023 ESC Cardiomyopathy
- NT-proBNP and congestion surveillance — 2022 ACC/AHA HF
- thrombus surveillance if AF or severe atrial enlargement — ESC 2024 AF
- family cascade if familial RCM — 2023 ESC Cardiomyopathy

Setting (outpatient) monitoring:
- Etiology-driven monitoring + serial echo/NPs — 2023 ESC Cardiomyopathy

Follow-up plan: Family cascade if familial; etiology-specific long-term care
- Close-out criterion: follow-up + cascade plan documented

Monitoring phase: Etiology-driven monitoring + congestion/AF/thrombus surveillance

Disposition

Current setting: outpatient — Resolve RCM-vs-CP, hunt + route etiology, manage congestion cautiously, anticoagulate AF (2023 ESC Cardiomyopathy)

Disposition criteria:
- Etiology identified → route to disease-specific engine + symptomatic co-management
- Constrictive pericarditis → pericardiectomy
- Idiopathic/familial end-stage → transplant evaluation

Escalation triggers (move to higher acuity):
- Constrictive pericarditis identified → surgical pericardiectomy — 2023 ESC Cardiomyopathy
- End-stage idiopathic/familial RCM → transplant — 2023 ESC Cardiomyopathy
- Decompensation → cautious acute pathway — 2022 ACC/AHA HF

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [SEVERE] Hemodynamics/imaging indicate constrictive pericarditis rather than RCM — pericardiectomy is potentially CURATIVE; do not mislabel RCM — 2023 ESC Cardiomyopathy
- [SEVERE] Amyloid as the RCM etiology (FLC/PYP) — route to ATTR or AL engine for disease-modifying therapy — Gillmore Circulation 2016
- [SEVERE] Fabry disease (low α-Gal in males / GLA variant) — enzyme replacement / migalastat; route metabolic pathway — 2023 ESC Cardiomyopathy

Citations

- 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; RCM-vs-constrictive-pericarditis hemodynamic literature [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- 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/)
- Cited evidence (PMID 32673028) [PMID:32673028](https://pubmed.ncbi.nlm.nih.gov/32673028/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline; RCM-vs-constrictive-pericarditis hemodynamic literature — PMID:37622657
  • Cited evidence (PMID 27143678) — PMID:27143678
  • Cited evidence (PMID 35379504) — PMID:35379504
  • Cited evidence (PMID 37622666) — PMID:37622666
  • Cited evidence (PMID 32673028) — PMID:32673028