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cardio.cardiac-sarcoidosis.chronic.v1PRODUCTION
cardio.cardiac-sarcoidosis.chronic.v1

Cardiac sarcoidosis (chronic — immunosuppression + arrhythmic protection)

cardiologychronicadult
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Care setting:

Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Suspect CS — especially young adult with unexplained high-grade AV block or non-ischemic VT

Inputs
2
Actions
0
Advance rule
Set
Advance when

CS clinically suspected

Patient inputs (11)

Patchy mid-wall/epicardial multi-territory LGE — CS imaging criterion

Active inflammation — directs + monitors immunosuppression

Young unexplained AV block strongly suggests CS

High-grade AV block — pacing + CS workup trigger

Systolic dysfunction → HFrEF GDMT + ICD criteria

Spontaneous sustained VT/VF = ICD (2014 HRS)

Functional status + GDMT/transplant timing

Immunosuppressant + GDMT dosing

Biopsy-proven extracardiac sarcoid supports clinical CS diagnosis

AF — rate/rhythm + anticoagulation

Myocardial injury/HF burden

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

Severity triggers (9)

9 need judgement
  • informationallife_threateningsustained_va_icd
    Spontaneous sustained VT/VF in CS — ICD (secondary prevention) — 2014 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninggiant_cell_myocarditis_overlap
    Fulminant, rapidly progressive course with VT/cardiogenic shock — consider giant-cell myocarditis (biopsy) — different urgent immunosuppression — 2014 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningtransplant_branch
    End-stage CS cardiomyopathy or intractable VT despite therapy — transplant evaluation (good outcomes; immunosuppression continues) — 2023 ESC Cardiomyopathy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereyoung_unexplained_av_block
    Unexplained high-grade AV block in adult <60 — CS until proven otherwise; advanced imaging (MRI/PET) BEFORE committing to a pacemaker-only device — 2014 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverefdg_pet_active_immunosuppression
    FDG-PET-active myocardial inflammation — corticosteroid immunosuppression to halt progression; titrate to serial PET — 2014 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresystolic_dysfunction_gdmt
    CS with LVEF ≤40 — standard HFrEF 4-pillar GDMT applies (granulomatous, not amyloid-stiff) — 2022 ACC/AHA HF
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_special_pop
    Pregnancy with CS — immunosuppressant selection (avoid methotrexate/mycophenolate; prednisone/azathioprine relatively safer), arrhythmia + device monitoring; cardio-obstetric — ESC 2018 Pregnancy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatesteroid_toxicity_branch
    Steroid toxicity / inability to taper — add steroid-sparing (methotrexate/azathioprine) or biologic; monitor glucose/bone/BP — 2014 HRS
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateckd_special_pop
    CKD — immunosuppressant + GDMT dose-gating; renal sarcoid co-involvement — KDIGO 2024
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

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Recommended regimen

Cardiac sarcoidosis — FDG-PET-guided immunosuppression + arrhythmic protection (2014 HRS; 2023 ESC Cardiomyopathy)
axis: cs_immunosuppression_and_arrhythmic_protectionstep 1 - Step 1 — Confirm CS + assess inflammation activity (FDG-PET) and arrhythmic risk
Selected step "Step 1 — Confirm CS + assess inflammation activity (FDG-PET) and arrhythmic risk" — Suspected CS (AV block / VT / patchy LGE / systemic sarcoid)

outpatient playbook — drug actions (3)

  1. 1. prednisone for PET-active inflammation
    30–40 mg/day then taper • PO • daily
    trigger: FDG-PET-active CS (2014 HRS)
    First-line immunosuppression
  2. 2. methotrexate/azathioprine steroid-sparing
    MTX 10–15 mg weekly • PO • weekly/daily
    trigger: Steroid taper / toxicity (2014 HRS)
    Steroid-sparing
  3. 3. pacemaker/ICD + AAD; HFrEF GDMT if low EF
    device / amiodarone / per HFrEF • device/PO • n/a
    trigger: AV block / VA / LVEF ≤35 (2014 HRS)
    Arrhythmic protection + HF therapy

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Unexplained high-grade AV block in adult <60 — CS until proven otherwise; Ventricular tachycardia / palpitations of unclear cause; CMR patchy non-ischemic LGE / FDG-PET focal myocardial uptake.

Objective

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

Assessment

**Cardiac sarcoidosis (chronic — immunosuppression + arrhythmic protection)** (cardio.cardiac-sarcoidosis.chronic.v1).
Phenotype framing: CS vs ARVC vs giant-cell myocarditis vs idiopathic VT/DCM vs amyloid
Scope: Suspect CS — especially young adult with unexplained high-grade AV block or non-ischemic VT

No severity triggers fired against current inputs.

Plan

Regimen axis: **Cardiac sarcoidosis — FDG-PET-guided immunosuppression + arrhythmic protection (2014 HRS; 2023 ESC Cardiomyopathy)** — step "Step 1 — Confirm CS + assess inflammation activity (FDG-PET) and arrhythmic risk".

Setting playbook (outpatient) — Confirm CS, FDG-PET-guide immunosuppression, protect against SCD/AV block (low ICD threshold), GDMT for systolic dysfunction (2014 HRS; 2023 ESC Cardiomyopathy)
1. prednisone for PET-active inflammation 30–40 mg/day then taper PO daily — FDG-PET-active CS (2014 HRS) (First-line immunosuppression)
2. methotrexate/azathioprine steroid-sparing MTX 10–15 mg weekly PO weekly/daily — Steroid taper / toxicity (2014 HRS) (Steroid-sparing)
3. pacemaker/ICD + AAD; HFrEF GDMT if low EF device / amiodarone / per HFrEF device/PO n/a — AV block / VA / LVEF ≤35 (2014 HRS) (Arrhythmic protection + HF therapy)

Non-pharmacologic actions:
- Cardiac-sarcoidosis centre + EP referral — 2014 HRS
- ICD-capable device when pacing indicated in CS — 2014 HRS
- Transplant evaluation for end-stage — 2023 ESC Cardiomyopathy

AVOID / contraindication checks:
- Young unexplained high grade AV block workup for CS before permanent pacing alone — 2014 HRS
- Use ICD capable device when pacing indicated in CS high VA risk — 2014 HRS
- Titrate immunosuppression to serial FDG PET activity — 2014 HRS
- Standard HFrEF GDMT applies in CS systolic dysfunction — 2022 ACC/AHA HF
- Consider giant cell myocarditis if fulminant rapidly progressive — 2014 HRS

Monitoring

Regimen monitoring:
- serial FDG PET to titrate immunosuppression — 2014 HRS
- device interrogation for AV block and VA — 2014 HRS
- serial LVEF and VA surveillance — 2014 HRS
- steroid toxicity surveillance glucose bone BP — 2014 HRS
- systemic sarcoid co management — 2023 ESC Cardiomyopathy

Setting (outpatient) monitoring:
- Serial FDG-PET to titrate immunosuppression — 2014 HRS
- Device + LVEF + VA surveillance; steroid toxicity — 2014 HRS

Follow-up plan: Steroid-sparing taper, relapse surveillance, systemic-sarcoid co-management
- Close-out criterion: long-term immunosuppression + relapse plan documented

Monitoring phase: Serial FDG-PET to titrate immunosuppression; device interrogation; LVEF/VA surveillance

Disposition

Current setting: outpatient — Confirm CS, FDG-PET-guide immunosuppression, protect against SCD/AV block (low ICD threshold), GDMT for systolic dysfunction (2014 HRS; 2023 ESC Cardiomyopathy)

Disposition criteria:
- PET-active → immunosuppression + device by risk + serial PET
- PET-inactive, low risk → surveillance ± maintenance steroid-sparing
- End-stage → transplant evaluation

Escalation triggers (move to higher acuity):
- Complete heart block / sustained VT → EP + ICD-capable device now — 2014 HRS
- Fulminant/rapidly progressive → consider giant-cell myocarditis, acute pathway — 2014 HRS
- End-stage cardiomyopathy / intractable VT → transplant — 2023 ESC Cardiomyopathy

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Spontaneous sustained VT/VF in CS — ICD (secondary prevention) — 2014 HRS
- [LIFE_THREATENING] Fulminant, rapidly progressive course with VT/cardiogenic shock — consider giant-cell myocarditis (biopsy) — different urgent immunosuppression — 2014 HRS
- [LIFE_THREATENING] End-stage CS cardiomyopathy or intractable VT despite therapy — transplant evaluation (good outcomes; immunosuppression continues) — 2023 ESC Cardiomyopathy

Citations

- 2014 HRS Cardiac Sarcoidosis Expert Consensus + 2023 ESC Cardiomyopathy Guideline + 2022 AHA/ACC/HFSA HF Guideline [PMID:24819193](https://pubmed.ncbi.nlm.nih.gov/24819193/)
- Cited evidence (PMID 37622657) [PMID:37622657](https://pubmed.ncbi.nlm.nih.gov/37622657/)
- 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 31535829) [PMID:31535829](https://pubmed.ncbi.nlm.nih.gov/31535829/)

Last reconciled with current guidelines: 2026-05-16.
References