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cardio.cardiac-sarcoidosis.chronic.v1

Cardiac sarcoidosis (chronic — immunosuppression + arrhythmic protection)

cardiologychronicadultoutpatienttransition

Cardiac sarcoidosis chronic — FDG-PET-guided immunosuppression (steroid → steroid-sparing → biologic) + low-threshold arrhythmic/SCD protection (2014 HRS: ICD-capable device when pacing indicated). Standard HFrEF GDMT applies for systolic dysfunction (granulomatous, not amyloid-stiff). Manifest points at existing sibling cardio.acute-hf.core.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (immunosuppression + arrhythmic + GDMT axis + workups + calculators + panels), test_files, 7-PMID evidence object, chronic phases all present. INTEGRATED (not PRODUCTION): prednisone/methotrexate/azathioprine/amiodarone/GDMT RxCUIs reused or standard; biologic/ablation/transplant/device non_pharm; SNOMED deferred. 9 trigger/special-pop branches: young AV block (not-to-miss), sustained-VA ICD, FDG-PET-active immunosuppression, giant-cell-myocarditis overlap, systolic-dysfunction GDMT, steroid toxicity, transplant, pregnancy, CKD.

Entry points (5)

  • lab_abnormality
    Unexplained high-grade AV block in adult <60 — CS until proven otherwise
    unexplained_high_grade_av_block
  • symptom
    Ventricular tachycardia / palpitations of unclear cause
    vt_or_palpitations
  • imaging
    CMR patchy non-ischemic LGE / FDG-PET focal myocardial uptake
    patchy_lge_or_pet_uptake
  • history
    Known systemic sarcoidosis with cardiac symptoms/ECG change
    known_systemic_sarcoidosis
  • symptom
    HF with regional wall-motion abnormality / aneurysm not matching coronary territory
    hf_with_regional_wma

Required inputs (11)

  • agerequired
    demographic • used at FRAME
    Young unexplained AV block strongly suggests CS
  • av_block_graderequired
    imaging • used at RED_FLAGS
    High-grade AV block — pacing + CS workup trigger
  • cmr_lge_patternrequired
    imaging • used at BRANCHING_WORKUP
    Patchy mid-wall/epicardial multi-territory LGE — CS imaging criterion
  • fdg_pet_activityrequired
    imaging • used at BRANCHING_WORKUP
    Active inflammation — directs + monitors immunosuppression
  • lvefrequired
    imaging • used at RISK_STRATIFICATION
    Systolic dysfunction → HFrEF GDMT + ICD criteria
  • extracardiac_sarcoid
    history • used at CONTEXT
    Biopsy-proven extracardiac sarcoid supports clinical CS diagnosis
  • sustained_varequired
    history • used at RISK_STRATIFICATION
    Spontaneous sustained VT/VF = ICD (2014 HRS)
  • troponin_nt_probnp
    lab • used at INITIAL_WORKUP
    Myocardial injury/HF burden
  • atrial_fibrillation
    history • used at CONTEXT
    AF — rate/rhythm + anticoagulation
  • creatininerequired
    lab • used at TREATMENT
    Immunosuppressant + GDMT dosing
  • nyha_classrequired
    symptom • used at RISK_STRATIFICATION
    Functional status + GDMT/transplant timing

12-phase flow (12)

  1. 1FRAME
    Suspect CS — especially young adult with unexplained high-grade AV block or non-ischemic VT
    inputs: age, av_block_grade
    advance: CS clinically suspected
  2. 2ENTRY
    AV block, VT, patchy LGE/PET uptake, known systemic sarcoid, regional WMA HF
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Systemic vs isolated CS, extracardiac biopsy status, AF
    inputs: extracardiac_sarcoid, atrial_fibrillation
    advance: systemic context complete
  4. 4RED_FLAGS
    Complete heart block, sustained VT/VF, decompensated HF
    inputs: av_block_grade, sustained_va
    actions: cardiogenic_shock
    advance: no red flags or routed to acute/EP pathway
  5. 5INITIAL_WORKUP
    ECG, echo (regional WMA/aneurysm), troponin/NT-proBNP
    inputs: troponin_nt_probnp
    actions: panel.cardiac
    advance: baseline cardiac assessment documented
  6. 6BRANCHING_WORKUP
    Cardiac MRI (LGE), FDG-PET (activity), extracardiac or endomyocardial biopsy, EP study; exclude giant-cell myocarditis if fulminant
    inputs: cmr_lge_pattern, fdg_pet_activity
    actions: preop_cardiac
    advance: CS diagnosis + inflammation activity established
  7. 7DIFFERENTIAL
    CS vs ARVC vs giant-cell myocarditis vs idiopathic VT/DCM vs amyloid
    inputs: cmr_lge_pattern, fdg_pet_activity
    advance: CS confirmed/staged (2014 HRS / 2016 JCS)
  8. 8RISK_STRATIFICATION
    2014 HRS ICD criteria (sustained VA, LVEF ≤35, pacing indication in CS, EP-inducible); PET activity; LVEF
    inputs: sustained_va, lvef, nyha_class
    advance: ICD + immunosuppression decisions assigned
  9. 9TREATMENT
    FDG-PET-guided immunosuppression (corticosteroid → steroid-sparing → biologic) + pacing/ICD (low threshold) + VT AAD/ablation + HFrEF GDMT if systolic dysfunction
    inputs: fdg_pet_activity, lvef, creatinine
    advance: immunosuppression + device + HF plan documented
  10. 10DISPOSITION
    Cardiac-sarcoidosis centre, EP, transplant evaluation for end-stage
    inputs: nyha_class
    actions: preop_cardiac
    advance: referral plan set
  11. 11MONITORING
    Serial FDG-PET to titrate immunosuppression; device interrogation; LVEF/VA surveillance
    inputs: fdg_pet_activity, lvef
    actions: panel.cardiac
    advance: monitoring cadence documented
  12. 12FOLLOWUP
    Steroid-sparing taper, relapse surveillance, systemic-sarcoid co-management
    inputs: fdg_pet_activity
    advance: long-term immunosuppression + relapse plan documented