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cardio.restrictive-cardiomyopathy.chronic.v1

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

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

RCM chronic — organised around the etiology hunt + the RCM-vs-constrictive-pericarditis pivot (CP curable by pericardiectomy). Symptomatic HF is amyloid-like (cautious preload-dependent decongestion; standard HFrEF GDMT generally not applicable unless dilated/systolic component). Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (etiology-routing + symptomatic axis + workups + calculators + panels), test_files, 7-PMID evidence object, chronic phases all present. INTEGRATED (not PRODUCTION): diuretic/MRA/DOAC/BB RxCUIs reused from validated cardio dossiers; etiology-specific therapies + pericardiectomy/transplant non_pharm/referral; SNOMED deferred. 9 trigger/special-pop branches: CP pivot, amyloid, Fabry, iron-overload, Loeffler/HES, idiopathic/familial, AF/atrial-thrombus, pregnancy, CKD.

Entry points (5)

  • imaging
    Echo: restrictive filling + biatrial enlargement + non-dilated ventricles + normal EF
    restrictive_filling_biatrial
  • symptom
    Right-heart congestion (edema, ascites, elevated JVP) with preserved EF
    right_heart_congestion
  • symptom
    Exertional dyspnea / fatigue out of proportion to EF
    exertional_dyspnea_fatigue
  • history
    Family history of RCM / known storage or infiltrative disease
    family_rcm_or_storage
  • history
    Mediastinal radiation / hypereosinophilia / carcinoid history
    radiation_or_eosinophilia

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Pediatric familial vs adult acquired; prognosis
  • restrictive_physiologyrequired
    imaging • used at INITIAL_WORKUP
    Restrictive Doppler/strain + biatrial enlargement defines the phenotype
  • rcm_vs_cp_hemodynamicsrequired
    imaging • used at BRANCHING_WORKUP
    Invasive hemodynamics (ventricular interdependence, respiratory discordance) — RCM vs constrictive pericarditis pivot
  • cmr_pattern
    imaging • used at BRANCHING_WORKUP
    CMR LGE pattern + pericardium — etiology + RCM-vs-CP
  • serum_free_light_chainsrequired
    lab • used at BRANCHING_WORKUP
    Amyloid screen (route AL/ATTR) — most common infiltrative RCM
  • ferritin_tsat
    lab • used at BRANCHING_WORKUP
    Iron-overload/hemochromatosis screen (T2* MRI if positive)
  • alpha_galactosidase
    lab • used at BRANCHING_WORKUP
    Fabry screen (males); GLA gene
  • eosinophil_count
    lab • used at BRANCHING_WORKUP
    Loeffler/hypereosinophilic endomyocardial disease
  • nt_probnprequired
    lab • used at RISK_STRATIFICATION
    Typically higher in RCM than constrictive pericarditis; prognosis
  • atrial_fibrillation
    history • used at CONTEXT
    AF with biatrial enlargement — high thrombus risk → anticoagulate
  • creatininerequired
    lab • used at TREATMENT
    Diuretic + drug dosing; cardiorenal in restrictive physiology
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Functional status + transplant timing

12-phase flow (12)

  1. 1FRAME
    Confirm restrictive physiology; immediately set up the RCM-vs-constrictive-pericarditis question (CP is curable)
    inputs: restrictive_physiology
    advance: restrictive physiology confirmed; CP question framed
  2. 2ENTRY
    Right-HF congestion, restrictive Doppler, biatrial enlargement, family/radiation/eosinophilia
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Etiology clues (family, radiation, eosinophils, iron, neuropathy), AF
    inputs: atrial_fibrillation, family_rcm_or_storage
    advance: etiology context catalogued
  4. 4RED_FLAGS
    Decompensated right HF, intracardiac thrombus, high-grade conduction block
    inputs: nyha_class
    actions: cardiogenic_shock, acute_pulm_edema
    advance: no red flags or routed to acute pathway
  5. 5INITIAL_WORKUP
    Echo (restrictive pattern, biatrial enlargement, EF), ECG, NT-proBNP
    inputs: restrictive_physiology, nt_probnp
    actions: panel.cardiac
    advance: restrictive phenotype quantified
  6. 6BRANCHING_WORKUP
    CMR (pericardium + LGE), invasive hemodynamics (RCM vs CP), etiology panel: FLC (→ amyloid), α-Gal/GLA (Fabry), ferritin/T2* (iron), eosinophils (Loeffler), genetics
    inputs: rcm_vs_cp_hemodynamics, serum_free_light_chains, ferritin_tsat, alpha_galactosidase, eosinophil_count
    actions: preop_cardiac
    advance: etiology assigned or routed; CP excluded/confirmed
  7. 7DIFFERENTIAL
    RCM etiologies (amyloid/Fabry/iron/Loeffler/idiopathic-familial) vs constrictive pericarditis
    inputs: rcm_vs_cp_hemodynamics, serum_free_light_chains
    advance: etiology + CP-status resolved
  8. 8RISK_STRATIFICATION
    Etiology-specific prognosis, atrial size/thrombus, NPs, transplant candidacy
    inputs: nt_probnp, nyha_class
    advance: prognosis + therapy routing assigned
  9. 9TREATMENT
    Etiology-specific routing (amyloid/Fabry/iron/HES/sarcoid) + cautious symptomatic HF (preload-dependent diuretic ± MRA; anticoagulate AF; avoid aggressive afterload reduction; HFrEF GDMT only if dilated/systolic component); pericardiectomy if CP
    inputs: creatinine
    advance: etiology routing + symptomatic plan documented
  10. 10DISPOSITION
    Route to etiology engines / pericardiectomy / transplant evaluation (idiopathic-familial poor prognosis)
    inputs: nyha_class
    actions: preop_cardiac
    advance: routing / referral plan set
  11. 11MONITORING
    Etiology-driven monitoring + congestion/AF/thrombus surveillance
    inputs: nt_probnp, creatinine
    actions: panel.cardiac
    advance: monitoring cadence documented
  12. 12FOLLOWUP
    Family cascade if familial; etiology-specific long-term care
    inputs: family_rcm_or_storage
    advance: follow-up + cascade plan documented