Cardiac sarcoidosis (chronic — immunosuppression + arrhythmic protection)
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_abnormalityUnexplained high-grade AV block in adult <60 — CS until proven otherwiseunexplained_high_grade_av_block
- symptomVentricular tachycardia / palpitations of unclear causevt_or_palpitations
- imagingCMR patchy non-ischemic LGE / FDG-PET focal myocardial uptakepatchy_lge_or_pet_uptake
- historyKnown systemic sarcoidosis with cardiac symptoms/ECG changeknown_systemic_sarcoidosis
- symptomHF with regional wall-motion abnormality / aneurysm not matching coronary territoryhf_with_regional_wma
Required inputs (11)
- agerequireddemographic • used at FRAMEYoung unexplained AV block strongly suggests CS
- av_block_graderequiredimaging • used at RED_FLAGSHigh-grade AV block — pacing + CS workup trigger
- cmr_lge_patternrequiredimaging • used at BRANCHING_WORKUPPatchy mid-wall/epicardial multi-territory LGE — CS imaging criterion
- fdg_pet_activityrequiredimaging • used at BRANCHING_WORKUPActive inflammation — directs + monitors immunosuppression
- lvefrequiredimaging • used at RISK_STRATIFICATIONSystolic dysfunction → HFrEF GDMT + ICD criteria
- extracardiac_sarcoidhistory • used at CONTEXTBiopsy-proven extracardiac sarcoid supports clinical CS diagnosis
- sustained_varequiredhistory • used at RISK_STRATIFICATIONSpontaneous sustained VT/VF = ICD (2014 HRS)
- troponin_nt_probnplab • used at INITIAL_WORKUPMyocardial injury/HF burden
- atrial_fibrillationhistory • used at CONTEXTAF — rate/rhythm + anticoagulation
- creatininerequiredlab • used at TREATMENTImmunosuppressant + GDMT dosing
- nyha_classrequiredsymptom • used at RISK_STRATIFICATIONFunctional status + GDMT/transplant timing
12-phase flow (12)
- 1FRAMESuspect CS — especially young adult with unexplained high-grade AV block or non-ischemic VTinputs: age, av_block_gradeadvance: CS clinically suspected
- 2ENTRYAV block, VT, patchy LGE/PET uptake, known systemic sarcoid, regional WMA HFinputs: ageadvance: entry trigger captured
- 3CONTEXTSystemic vs isolated CS, extracardiac biopsy status, AFinputs: extracardiac_sarcoid, atrial_fibrillationadvance: systemic context complete
- 4RED_FLAGSComplete heart block, sustained VT/VF, decompensated HFinputs: av_block_grade, sustained_vaactions: cardiogenic_shockadvance: no red flags or routed to acute/EP pathway
- 5INITIAL_WORKUPECG, echo (regional WMA/aneurysm), troponin/NT-proBNPinputs: troponin_nt_probnpactions: panel.cardiacadvance: baseline cardiac assessment documented
- 6BRANCHING_WORKUPCardiac MRI (LGE), FDG-PET (activity), extracardiac or endomyocardial biopsy, EP study; exclude giant-cell myocarditis if fulminantinputs: cmr_lge_pattern, fdg_pet_activityactions: preop_cardiacadvance: CS diagnosis + inflammation activity established
- 7DIFFERENTIALCS vs ARVC vs giant-cell myocarditis vs idiopathic VT/DCM vs amyloidinputs: cmr_lge_pattern, fdg_pet_activityadvance: CS confirmed/staged (2014 HRS / 2016 JCS)
- 8RISK_STRATIFICATION2014 HRS ICD criteria (sustained VA, LVEF ≤35, pacing indication in CS, EP-inducible); PET activity; LVEFinputs: sustained_va, lvef, nyha_classadvance: ICD + immunosuppression decisions assigned
- 9TREATMENTFDG-PET-guided immunosuppression (corticosteroid → steroid-sparing → biologic) + pacing/ICD (low threshold) + VT AAD/ablation + HFrEF GDMT if systolic dysfunctioninputs: fdg_pet_activity, lvef, creatinineadvance: immunosuppression + device + HF plan documented
- 10DISPOSITIONCardiac-sarcoidosis centre, EP, transplant evaluation for end-stageinputs: nyha_classactions: preop_cardiacadvance: referral plan set
- 11MONITORINGSerial FDG-PET to titrate immunosuppression; device interrogation; LVEF/VA surveillanceinputs: fdg_pet_activity, lvefactions: panel.cardiacadvance: monitoring cadence documented
- 12FOLLOWUPSteroid-sparing taper, relapse surveillance, systemic-sarcoid co-managementinputs: fdg_pet_activityadvance: long-term immunosuppression + relapse plan documented