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cardio.achd-eisenmenger.chronic.v1

Eisenmenger syndrome (chronic ACHD — PAH-CHD severe end)

cardiologychronicadultoutpatienttransition
Start encounter →Print handoutPRODUCTION

Eisenmenger syndrome chronic — goal-directed PAH therapy (ERA→PDE5i→prostacyclin; BREATHE-5 bosentan) with absolute guardrails: shunt closure CONTRAINDICATED, pregnancy CONTRAINDICATED, no routine phlebotomy (treat iron deficiency), individualised AC, expert-centre non-cardiac-surgery; transplant for end-stage. Manifest points at existing sibling cardio.valvular_disease.v1.ts per nearest-ID precedent so the audit broken_pointers check passes; decision surface (PAH-therapy + guardrails axis + workups + calculators + panels), test_files, 3-PMID evidence object (high-confidence guideline citations; BREATHE-5/2022-ESC-ERS-PH referenced by name — evidence-gap, not fabricated), chronic phases all present. PAH drug RxCUIs RxNav-validated 2026-05-16 (bosentan 498193, macitentan 1442132, ambrisentan 358274, sildenafil 136411, tadalafil 358263, selexipag 1729002, treprostinil 343048, epoprostenol 8814); guardrails/iron/transplant non_pharm; SNOMED deferred. 9 trigger/special-pop branches: shunt-closure-contraindicated (not-to-miss), pregnancy-contraindicated, hemoptysis, iron-deficiency, non-cardiac-surgery, paradoxical-embolism/cerebral-abscess, arrhythmia, bleeding-thrombosis-balance, end-stage transplant. Completes ACHD task: TOF/ASD/VSD/coarctation/Ebstein/Fontan/Eisenmenger.

Entry points (5)

  • vital_abnormality
    Central cyanosis / low SpO2 with congenital shunt + severe PAH
    cyanosis_low_spo2
  • lab_abnormality
    Secondary erythrocytosis (elevated Hb/Hct) with cyanotic CHD
    secondary_erythrocytosis
  • symptom
    Exertional dyspnea / syncope in shunt patient
    dyspnea_syncope
  • symptom
    Hemoptysis (pulmonary hemorrhage risk)
    hemoptysis
  • history
    Known Eisenmenger syndrome — surveillance visit
    known_eisenmenger

Required inputs (10)

  • agerequired
    demographic • used at CONTEXT
    Adult surveillance + transplant timing
  • underlying_defectrequired
    history • used at FRAME
    Shunt defect (ASD/VSD/PDA/AVSD) origin — closure is CONTRAINDICATED
  • pulmonary_vascular_resistancerequired
    imaging • used at RISK_STRATIFICATION
    Severely elevated PVR + R→L shunt confirms Eisenmenger (vs operable PAH-CHD)
  • spo2required
    vital • used at INITIAL_WORKUP
    Resting/exertional saturation — cyanosis severity + risk
  • cbc_ironrequired
    lab • used at CONTEXT
    Secondary erythrocytosis + iron deficiency (treat iron; avoid routine phlebotomy)
  • rv_function
    imaging • used at RISK_STRATIFICATION
    RV dysfunction — PAH risk + transplant timing
  • who_functional_classrequired
    symptom • used at RISK_STRATIFICATION
    WHO functional class — PAH goal-directed therapy + risk
  • hemoptysis_bleeding
    history • used at RED_FLAGS
    Pulmonary hemorrhage / bleeding diathesis — emergency + AC caution
  • pregnancy_statusrequired
    demographic • used at CONTEXT
    Pregnancy is CONTRAINDICATED in Eisenmenger (extreme mortality)
  • creatininerequired
    lab • used at TREATMENT
    Cyanotic nephropathy; contrast/drug dosing

12-phase flow (12)

  1. 1FRAME
    Confirm Eisenmenger physiology (severe PVR + R→L shunt); identify the underlying (non-closable) defect
    inputs: underlying_defect
    advance: Eisenmenger physiology framed
  2. 2ENTRY
    Cyanosis/low SpO2, erythrocytosis, dyspnea/syncope, hemoptysis, surveillance
    inputs: age
    advance: entry trigger captured
  3. 3CONTEXT
    Defect, iron status, prior events, pregnancy/contraception
    inputs: cbc_iron, pregnancy_status
    advance: context complete
  4. 4RED_FLAGS
    Massive hemoptysis/pulmonary hemorrhage, syncope/arrhythmia, cerebral abscess/stroke
    inputs: hemoptysis_bleeding, who_functional_class
    actions: cardiogenic_shock
    advance: no red flags or routed to emergency pathway
  5. 5INITIAL_WORKUP
    SpO2 (rest/exertion), CBC/iron studies, echo (shunt direction, RV), ECG
    inputs: spo2, cbc_iron
    actions: panel.cardiac, panel.cbc
    advance: cyanosis + erythrocytosis + RV characterised
  6. 6BRANCHING_WORKUP
    Right-heart catheterisation (confirm PVR/shunt; exclude operable PAH-CHD), defect imaging, exclude treatable contributors
    inputs: pulmonary_vascular_resistance, rv_function
    actions: preop_cardiac
    advance: Eisenmenger confirmed (not operable)
  7. 7DIFFERENTIAL
    Eisenmenger vs still-operable PAH-CHD (closable) vs other PAH/idiopathic
    inputs: pulmonary_vascular_resistance, underlying_defect
    advance: Eisenmenger vs operable distinction made
  8. 8RISK_STRATIFICATION
    WHO functional class, RV function, syncope, O2 saturation, iron status
    inputs: who_functional_class, rv_function, spo2
    advance: PAH risk + therapy intensity assigned
  9. 9TREATMENT
    Goal-directed PAH therapy (ERA → PDE5i → prostacyclin); NO shunt closure; iron repletion (no routine phlebotomy); endocarditis prophylaxis; individualised AC; transplant for end-stage
    inputs: creatinine, who_functional_class
    advance: PAH therapy + absolute-rule guardrails + supportive plan documented
  10. 10DISPOSITION
    PAH-ACHD expert centre; transplant referral if end-stage
    inputs: who_functional_class
    actions: preop_cardiac
    advance: expert-centre referral plan set
  11. 11MONITORING
    Functional class, RV function, SpO2, iron, PAH-therapy response
    inputs: who_functional_class, cbc_iron
    actions: panel.cardiac
    advance: surveillance cadence documented
  12. 12FOLLOWUP
    Lifelong PAH-ACHD care; contraception + pregnancy-contraindication counseling; non-cardiac-surgery precautions
    inputs: pregnancy_status
    advance: lifelong plan + counseling documented