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cardio.stemi.sickle-cell-vaso-occlusion.v1

STEMI in sickle cell disease (vaso-occlusive / microvascular)

cardiologyacuteadultacuteinpatienttransitionoutpatient
Start encounter →Print handoutPRODUCTION

Phase E variant of cardio.stemi.core.v1 — STEMI in sickle cell disease with vaso-occlusive crisis or microvascular pattern (HbSS most severe; HbSC, HbS-β-thal). Inherits reperfusion + antiplatelet + statin + BB regimen from parent via routing; specializes for: emergent cath often shows MINOCA pattern (cardiac MRI confirms microvascular MI), CAUTIOUS standard ACS bundle (anticoagulation bleed risk elevated with concurrent VOC + rhabdomyolysis), automated red cell EXCHANGE TRANSFUSION targeting HbS <30%, aggressive isotonic hydration (avoid over-hydration → ACS), opioid pain control via PCA, oxygen for SpO2 ≥95%, long-term hydroxyurea + L-glutamine + voxelotor + crizanlizumab per NHLBI 2014 + ASH 2020. Manifest pointer reuses cardio.stemi.core.v1 manifest. Design-brief pointer reuses parent (SCD-specific differences documented inline). Status INTEGRATED. Authored 2026-05-15 by shard-06-cardio-acute as Phase E rare-etiology variant.

Entry points (3)

  • history
    Known SCD (HbSS, HbSC, HbS-β-thal) presenting with chest pain → STEMI/NSTEMI workup with VOC + ACS overlap consideration
    sickle_cell_disease_with_chest_pain
  • imaging
    ST-elevation pattern on ECG in SCD patient — emergent cath; expect microvascular pattern more often than obstructive plaque
    ecg_st_elevation_in_scd_patient
  • symptom
    Vaso-occlusive crisis + chest pain + troponin elevation — overlaps with ACS, acute chest syndrome, microvascular MI
    voc_with_chest_pain_and_troponin

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    SCD-related ACS often presents younger than de novo ASCVD; presentations in pregnancy + post-op + dehydration triggers
  • scd_genotype_and_baseline_hbs_percentrequired
    history • used at CONTEXT
    HbSS most severe; baseline HbS% guides exchange transfusion target (<30%)
  • voc_trigger_historyrequired
    history • used at CONTEXT
    Identify reversible triggers — dehydration, infection, pregnancy, post-op, hypoxia, cold exposure
  • sbprequired
    vital • used at RED_FLAGS
    Hypotension may indicate sepsis (functional asplenia → encapsulated organism risk), splenic sequestration, or true cardiogenic compromise
  • spo2required
    vital • used at RED_FLAGS
    Hypoxia must be corrected aggressively — perpetuates sickling; low threshold for supplemental O2 even at "normal" SpO2 if symptomatic
  • ecgrequired
    imaging • used at INITIAL_WORKUP
    Standard STEMI criteria; SCD patients may have baseline LVH from chronic anemia
  • troponinrequired
    lab • used at INITIAL_WORKUP
    Standard ACS biomarker; in SCD microvascular MI may have modest elevation; serial trending essential
  • creatininerequired
    lab • used at CONTEXT
    SCD nephropathy common; baseline AKI from VOC/rhabdomyolysis must be assessed before contrast
  • hemolysis_panel_ldh_haptoglobin_reticsrequired
    lab • used at INITIAL_WORKUP
    Baseline + acute hemolysis markers; hyperhemolysis screening; informs exchange transfusion urgency
  • cor_angio_with_cardiac_mri_followuprequired
    imaging • used at TREATMENT
    Emergent cath to exclude obstructive disease; expect MINOCA pattern (non-obstructive); cardiac MRI critical for confirming microvascular MI vs myocarditis vs Takotsubo

12-phase flow (10)

  1. 1FRAME
    STEMI/NSTEMI in SCD = often microvascular MINOCA pattern from vaso-occlusion + endothelial dysfunction; route to cardio.stemi.core.v1 reperfusion arc but flag SCD-specific exchange transfusion + hydration + pain control
    inputs: ecg, scd_genotype_and_baseline_hbs_percent
    advance: STEMI confirmed + SCD context elicited
  2. 2ENTRY
    Cath lab within 90 min — but parallel-track exchange transfusion preparation, aggressive hydration, opioid pain control, oxygen
    inputs: age, spo2
    advance: cath lab activated + hematology consult notified
  3. 3CONTEXT
    SCD genotype + baseline HbS% + VOC trigger history + chronic SCD therapy (hydroxyurea, voxelotor, crizanlizumab); allergies; bleed risk; renal function
    inputs: sbp, creatinine, voc_trigger_history
    advance: SCD context + triggers documented
  4. 4RED_FLAGS
    Acute chest syndrome (new infiltrate + chest pain + fever/hypoxia — often co-exists with VOC); hyperhemolysis; splenic sequestration; sepsis (functional asplenia); stroke (silent + overt)
    inputs: sbp, spo2
    actions: cardiogenic_shock
    advance: acute chest syndrome + sepsis + stroke screened
  5. 5INITIAL_WORKUP
    ECG + troponin + BMP + CBC + retic + LDH + haptoglobin + CXR (acute chest syndrome screen) + bedside echo (LV function + RV strain + chronic anemia LVH)
    inputs: ecg, troponin, creatinine, hemolysis_panel_ldh_haptoglobin_retics
    actions: acs_pathway, panel.cardiac, panel.renal
    advance: workup + hemolysis baseline documented
  6. 6BRANCHING_WORKUP
    Emergent cath to exclude obstructive disease — expect MINOCA pattern; if obstructive: standard PCI; if non-obstructive: cardiac MRI to confirm microvascular MI; concurrent automated red cell exchange transfusion
    inputs: cor_angio_with_cardiac_mri_followup
    advance: cath complete + MRI booked + exchange transfusion started if non-obstructive
  7. 7TREATMENT
    Standard ACS regimen (ASA + ticagrelor + UFH + statin + BB) per cardio.stemi.core.v1 — but CAUTIOUSLY: anticoagulation bleed risk elevated with concurrent VOC + rhabdomyolysis. ADD: automated red cell EXCHANGE TRANSFUSION targeting HbS <30%; aggressive isotonic hydration (avoid over-hydration → ACS); opioid pain control (PCA morphine/hydromorphone — fentanyl alternative if renal); oxygen to SpO2 ≥95%; treat triggers (sepsis, dehydration)
    inputs: sbp, creatinine
    actions: protocol.stemi
    advance: reperfusion + exchange transfusion + supportive care started
  8. 8DISPOSITION
    CICU post-cath; hematology + SCD specialist consult; transition to chronic SCD-modifying therapy
    advance: unit assigned + hematology plan documented
  9. 9MONITORING
    Telemetry; serial CBC + retic + LDH (hemolysis trending); CXR for acute chest syndrome evolution; opioid effect + sedation; renal function trending; HbS% post-exchange
    inputs: hemolysis_panel_ldh_haptoglobin_retics
    actions: panel.cardiac
    advance: hemolysis controlled + HbS <30%
  10. 10FOLLOWUP
    Cardiology + hematology dual follow-up; initiate or up-titrate hydroxyurea (NHLBI Class I); consider L-glutamine, voxelotor, crizanlizumab; transcranial Doppler stroke screen (children + repeat in adults); SCD specialty clinic enrollment
    advance: chronic SCD therapy + cardiology bundle in place