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cardio.stemi.kawasaki-related.v1PRODUCTION
cardio.stemi.kawasaki-related.v1

STEMI from prior Kawasaki disease coronary aneurysm / stenosis

cardiologyacuteadult
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10/12 authored

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

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Detailed

KD-related STEMI = thrombosis or stenosis of a persistent post-KD coronary aneurysm in a young adult. Standard ACS reperfusion paradigm applies, BUT aneurysm anatomy changes PCI feasibility and long-term anticoagulation strategy.

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KD-related etiology confirmed

Patient inputs (10)

KD-related STEMI typically presents in 20-40s — much younger than atherosclerotic ACS; raises pre-test probability for non-atherosclerotic cause

Contrast nephropathy risk for cath + DOAC dosing

Documented childhood KD (with or without timely IVIG) is the central diagnostic anchor; aneurysm risk: 15-25% if untreated, <5% if IVIG within 10 days

Prior echo / cardiac MRI / CTA / cath imaging shows whether aneurysms persist, their size (giant ≥8 mm or Z-score ≥10), and which arteries — directly drives PCI vs CABG decision

ST elevation territory localizes culprit aneurysm / stenosis

Quantifies infarct burden + drives emergency reperfusion decision

LVEF + regional wall motion + aneurysm visualization (where coronary anatomy permits)

Hypotension + STEMI in giant aneurysm patient → cardiogenic shock high probability

Diagnostic + therapeutic gold standard; aneurysm anatomy + thrombus burden + multivessel involvement determines PCI vs CABG

TEE if giant aneurysm with concern for endocarditis (rare) or to characterize thrombus extent

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

Severity triggers (4)

4 need judgement
  • informationallife_threateningkawasaki_giant_aneurysm_with_extensive_thrombus
    Coronary angiogram or CTA showing giant aneurysm (≥8 mm or Z-score ≥10) with extensive intra-aneurysmal thrombus + STEMI
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningkawasaki_multi_aneurysm_requiring_cabg
    Multivessel coronary involvement with multiple aneurysms where PCI is technically infeasible → CABG required
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningkawasaki_sudden_cardiac_death_risk_after_event
    EF <35 + non-sustained VT on telemetry post-KD-STEMI → SCD risk in 40-90 d window
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverekawasaki_pediatric_to_adult_care_transition_failure
    Young adult with KD history presenting acutely without established adult congenital cardiology care; never transitioned from pediatric care
    Trigger could not be auto-evaluated — needs clinician judgement.

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RISK_STRATIFICATIONoptionalDrives risk stratification
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Recommended regimen

Kawasaki disease coronary aneurysm + STEMI regimen — combines parent acute reperfusion with lifelong aneurysm-thrombosis prevention; triple therapy if persistent giant aneurysm with thrombus
axis: kawasaki_aneurysm_thrombosis_phenotype
Selected axis "Kawasaki disease coronary aneurysm + STEMI regimen — combines parent acute reperfusion with lifelong aneurysm-thrombosis prevention; triple therapy if persistent giant aneurysm with thrombus" by default fallback (first axis)
  • aspirin
    first line
    antiplatelet_cox1
    162-325 mg chewed load → 81 mg daily lifelong • PO • daily indefinitely
    triggers: kawasaki_stemi_confirmed
    AHA Kawasaki 2024 (PMID 38683866) — ASA lifelong if persistent aneurysm; AHA 2025 ACS Class I for STEMI
    rxcui 243670
  • ticagrelor
    first line
    p2y12_inhibitor
    180 mg load → 90 mg BID • PO • BID × 12 mo standard DAPT, then reassess
    triggers: kawasaki_stemi_pci_planned
    PLATO PMID 19717846; standard DAPT post-PCI; duration may extend beyond 12 mo if persistent aneurysm thrombus
    rxcui 1116632
  • unfractionated heparin
    first line
    anticoagulant_indirect
    70-100 U/kg IV bolus + activated infusion • IV • bolus + infusion at PCI; transition to long-term AC if giant aneurysm
    triggers: kawasaki_stemi_pci_planned, aneurysm_thrombus_present
    AHA 2025 Class I for PCI; AHA Kawasaki 2024 — AC indicated for giant aneurysm thrombus
    rxcui 5224
  • warfarin
    comorbidity specific
    vitamin_k_antagonist
    5 mg daily; INR target 2-3 indefinitely if persistent giant aneurysm with thrombus • PO • daily indefinitely if giant aneurysm + thrombus
    triggers: persistent_giant_aneurysm_with_thrombus_post_event, kawasaki_aneurysm_8mm_or_z_score_above_10
    AHA Kawasaki 2024 (PMID 38683866) Class I lifelong AC for giant aneurysm with thrombus; warfarin preferred over DOAC due to limited DOAC RCT data in this population
    rxcui 11289
  • apixaban
    second line
    doac_factor_xa_direct
    5 mg BID (or 2.5 mg BID per dose-reduction criteria) • PO • BID
    triggers: warfarin_intolerant_or_INR_unstable, persistent_giant_aneurysm_with_thrombus
    AHA Kawasaki 2024 — DOAC alternative if warfarin contraindicated; off-label-but-rational; small case series support use
    rxcui 1364430
  • atorvastatin
    first line
    statin_high_intensity
    40-80 mg daily lifelong • PO • daily
    triggers: kawasaki_stemi_confirmed
    AHA Kawasaki 2024 — statin lifelong post-coronary-event regardless of LDL; PROVE-IT framework + KD-specific endothelial dysfunction rationale
    rxcui 83367
  • carvedilol
    first line
    beta_blocker_nonselective
    3.125 mg BID titrate • PO • BID
    triggers: kawasaki_stemi_with_lv_dysfunction, ef_below_40
    CAPRICORN PMID 11356436 — post-MI BB benefit; carvedilol preferred for HFrEF GDMT
    rxcui 20352
  • sacubitril-valsartan
    add on
    arni
    24/26 mg BID titrate • PO • BID
    triggers: kawasaki_stemi_with_ef_below_40_post_event
    PIONEER-HF PMID 30403955; ACC/AHA 2022 HF Class I if HFrEF persists post-MI
    rxcui 1656328

outpatient playbook — drug actions (1)

  1. 1. continue lifelong bundle
    rxcui 243670
    ASA 81 + warfarin INR 2-3 (if giant aneurysm) + atorvastatin 80 + GDMT if HFrEF • PO • as scheduled
    trigger: KD-related coronary disease lifelong
    AHA Kawasaki 2024

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Young adult (20-40s) with documented childhood Kawasaki disease history presenting with chest pain; ST elevation on ECG in patient with known persistent coronary aneurysm from KD — emergent cath; Incidental giant coronary aneurysm finding on cardiac imaging in adult presenting with ACS — KD aneurysm presumed.

Objective

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

Assessment

**STEMI from prior Kawasaki disease coronary aneurysm / stenosis** (cardio.stemi.kawasaki-related.v1).
Scope: KD-related STEMI = thrombosis or stenosis of a persistent post-KD coronary aneurysm in a young adult. Standard ACS reperfusion paradigm applies, BUT aneurysm anatomy changes PCI feasibility and long-term anticoagulation strategy.

No severity triggers fired against current inputs.

Plan

Regimen axis: **Kawasaki disease coronary aneurysm + STEMI regimen — combines parent acute reperfusion with lifelong aneurysm-thrombosis prevention; triple therapy if persistent giant aneurysm with thrombus**.
1. aspirin 162-325 mg chewed load → 81 mg daily lifelong PO daily indefinitely (antiplatelet_cox1, first line) — AHA Kawasaki 2024 (PMID 38683866) — ASA lifelong if persistent aneurysm; AHA 2025 ACS Class I for STEMI
2. ticagrelor 180 mg load → 90 mg BID PO BID × 12 mo standard DAPT, then reassess (p2y12_inhibitor, first line) — PLATO PMID 19717846; standard DAPT post-PCI; duration may extend beyond 12 mo if persistent aneurysm thrombus
3. unfractionated heparin 70-100 U/kg IV bolus + activated infusion IV bolus + infusion at PCI; transition to long-term AC if giant aneurysm (anticoagulant_indirect, first line) — AHA 2025 Class I for PCI; AHA Kawasaki 2024 — AC indicated for giant aneurysm thrombus
4. warfarin 5 mg daily; INR target 2-3 indefinitely if persistent giant aneurysm with thrombus PO daily indefinitely if giant aneurysm + thrombus (vitamin_k_antagonist, comorbidity specific) — AHA Kawasaki 2024 (PMID 38683866) Class I lifelong AC for giant aneurysm with thrombus; warfarin preferred over DOAC due to limited DOAC RCT data in this population
5. apixaban 5 mg BID (or 2.5 mg BID per dose-reduction criteria) PO BID (doac_factor_xa_direct, second line) — AHA Kawasaki 2024 — DOAC alternative if warfarin contraindicated; off-label-but-rational; small case series support use
6. atorvastatin 40-80 mg daily lifelong PO daily (statin_high_intensity, first line) — AHA Kawasaki 2024 — statin lifelong post-coronary-event regardless of LDL; PROVE-IT framework + KD-specific endothelial dysfunction rationale
7. carvedilol 3.125 mg BID titrate PO BID (beta_blocker_nonselective, first line) — CAPRICORN PMID 11356436 — post-MI BB benefit; carvedilol preferred for HFrEF GDMT
8. sacubitril-valsartan 24/26 mg BID titrate PO BID (arni, add on) — PIONEER-HF PMID 30403955; ACC/AHA 2022 HF Class I if HFrEF persists post-MI

Setting playbook (outpatient) — Lifelong adult congenital cardiology surveillance: serial CTA every 1-3 yr for stable aneurysm; lifetime triple-therapy management; continued GDMT if HFrEF; transplant evaluation if severe LV dysfunction; pregnancy + family planning counseling
9. continue lifelong bundle ASA 81 + warfarin INR 2-3 (if giant aneurysm) + atorvastatin 80 + GDMT if HFrEF PO as scheduled — KD-related coronary disease lifelong (AHA Kawasaki 2024)

Non-pharmacologic actions:
- Pregnancy counseling: high-risk obstetrics + cardiology team if pregnancy planned (warfarin teratogenic — switch to LMWH)
- Lifelong cardiac rehab maintenance
- Mental health continuity (chronic disease + young adult adjustment)
- Driving restriction if VF arrest occurred during event

AVOID / contraindication checks:
- Warfarin_avoid_active_bleeding (AHA 2022)
- Apixaban_avoid_severe_renal_impairment_egfr_below_25 (drug label)
- Warfarin_INR_target_2 3_for_aneurysm_thrombus (AHA Kawasaki 2024)
- Tenecteplase_avoid_in_kd_aneurysm_unless_pci_unavailable (aneurysm rupture concern — limited data)
- Lifelong_triple_therapy_bleeding_risk_assessment (case by case)

Monitoring

Regimen monitoring:
- echo at 5-7d post event for thrombus screen
- coronary CTA at 4-6 weeks post event to reassess aneurysm anatomy
- INR q week during warfarin initiation then q month long term
- serial coronary CTA q 1-3 yr for stable aneurysm lifelong (AHA Kawasaki 2024 — CTA preferred over invasive cath in stable patients)

Setting (outpatient) monitoring:
- Coronary CTA every 1-3 yr
- Annual lipid + BP + INR + BMP

Follow-up plan: Lifetime ASA + statin; long-term AC if persistent giant aneurysm with thrombus; serial coronary CTA (preferred over invasive cath in stable patient); transplant referral if severe LV dysfunction; pregnancy + family planning counseling (for women); transition pediatric → adult congenital cardiology
- Close-out criterion: long-term surveillance + congenital cardiology handoff complete

Monitoring phase: Telemetry, daily exam, echo at 5-7 d for thrombus / new aneurysm; coronary CTA at 4-6 weeks to reassess aneurysm anatomy post-event

Disposition

Current setting: outpatient — Lifelong adult congenital cardiology surveillance: serial CTA every 1-3 yr for stable aneurysm; lifetime triple-therapy management; continued GDMT if HFrEF; transplant evaluation if severe LV dysfunction; pregnancy + family planning counseling

Disposition criteria:
- Long-term continuation; cross-link to cardio.hf.core.v1 if HFrEF persists

Escalation triggers (move to higher acuity):
- New aneurysm or aneurysm enlargement on CTA → adult congenital cardiology + interventional consultation
- EF declining despite GDMT → advanced HF / transplant evaluation
- Bleeding on triple therapy → reassess regimen

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Coronary angiogram or CTA showing giant aneurysm (≥8 mm or Z-score ≥10) with extensive intra-aneurysmal thrombus + STEMI
- [LIFE_THREATENING] Multivessel coronary involvement with multiple aneurysms where PCI is technically infeasible → CABG required
- [LIFE_THREATENING] EF <35 + non-sustained VT on telemetry post-KD-STEMI → SCD risk in 40-90 d window

Citations

- AHA Kawasaki disease 2024 statement + JCS 2020 Kawasaki adult cardiac sequelae + 2025 ACC/AHA ACS Guideline + Kato Circulation 1996 long-term follow-up [PMID:38683866](https://pubmed.ncbi.nlm.nih.gov/38683866/)
- Cited evidence (PMID 37622670) [PMID:37622670](https://pubmed.ncbi.nlm.nih.gov/37622670/)
- Cited evidence (PMID 35718438) [PMID:35718438](https://pubmed.ncbi.nlm.nih.gov/35718438/)
- Cited evidence (PMID 38587234) [PMID:38587234](https://pubmed.ncbi.nlm.nih.gov/38587234/)
- Cited evidence (PMID 11907286) [PMID:11907286](https://pubmed.ncbi.nlm.nih.gov/11907286/)

Last reconciled with current guidelines: 2026-05-15.
References
  • AHA Kawasaki disease 2024 statement + JCS 2020 Kawasaki adult cardiac sequelae + 2025 ACC/AHA ACS Guideline + Kato Circulation 1996 long-term follow-up — PMID:38683866
  • Cited evidence (PMID 37622670) — PMID:37622670
  • Cited evidence (PMID 35718438) — PMID:35718438
  • Cited evidence (PMID 38587234) — PMID:38587234
  • Cited evidence (PMID 11907286) — PMID:11907286