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cardio.achd-eisenmenger.chronic.v1PRODUCTION
cardio.achd-eisenmenger.chronic.v1

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

cardiologychronicadult
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12/12 authored

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

Current phase

Frame

Detailed

Confirm Eisenmenger physiology (severe PVR + R→L shunt); identify the underlying (non-closable) defect

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Eisenmenger physiology framed

Patient inputs (10)

Adult surveillance + transplant timing

Secondary erythrocytosis + iron deficiency (treat iron; avoid routine phlebotomy)

Pregnancy is CONTRAINDICATED in Eisenmenger (extreme mortality)

Shunt defect (ASD/VSD/PDA/AVSD) origin — closure is CONTRAINDICATED

Resting/exertional saturation — cyanosis severity + risk

Severely elevated PVR + R→L shunt confirms Eisenmenger (vs operable PAH-CHD)

WHO functional class — PAH goal-directed therapy + risk

Cyanotic nephropathy; contrast/drug dosing

Pulmonary hemorrhage / bleeding diathesis — emergency + AC caution

RV dysfunction — PAH risk + transplant timing

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

Severity triggers (9)

9 need judgement
  • informationallife_threateningshunt_closure_contraindicated
    Eisenmenger physiology (severe PVR + R→L shunt) — shunt closure is CONTRAINDICATED (fatal); do not refer for defect closure — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningpregnancy_contraindicated
    Pregnancy in Eisenmenger — CONTRAINDICATED (maternal mortality ~30–50%); urgent expert counseling, contraception, termination discussion — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninghemoptysis_pulmonary_hemorrhage
    Hemoptysis / pulmonary hemorrhage — potentially life-threatening; expert evaluation, reverse/avoid anticoagulation, imaging — 2022 ESC/ERS PH
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningnon_cardiac_surgery_branch
    Any non-cardiac surgery / anesthesia — very high perioperative mortality; expert-centre management, avoid systemic vasodilation/hypovolemia, air-filter precautions — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningend_stage_transplant
    Refractory WHO FC III–IV despite goal-directed therapy — heart-lung or lung+repair transplant evaluation — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereparadoxical_embolism_cerebral_abscess
    Right-to-left shunt → paradoxical embolism / cerebral abscess risk — meticulous IV air filters; fever/neuro symptoms → urgent imaging — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverearrhythmia_poorly_tolerated
    Atrial/ventricular arrhythmia — poorly tolerated in Eisenmenger; expert rhythm management (avoid pro-arrhythmic destabilisation) — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateiron_deficiency_branch
    Iron deficiency in cyanotic erythrocytosis — repletion improves symptoms; do NOT routinely phlebotomize compensated erythrocytosis — 2018 AHA/ACC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatebleeding_thrombosis_balance
    Anticoagulation decision — individualised: hemoptysis/bleeding diathesis vs intrapulmonary/paradoxical thrombosis; NOT routine, not CHA₂DS₂-VASc-driven alone — 2020 ESC ACHD
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Eisenmenger — goal-directed PAH therapy + absolute-rule guardrails (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)
axis: eisenmenger_pah_therapy_and_guardrailsstep 1 - Step 1 — Absolute-rule guardrails (every Eisenmenger patient)
Selected step "Step 1 — Absolute-rule guardrails (every Eisenmenger patient)" — Confirmed Eisenmenger syndrome
  • do NOT close the shunt; pregnancy CONTRAINDICATED (contraception counseling); avoid routine phlebotomy/dehydration/high-altitude/iatrogenic air emboli
    first line
    safety_guardrail
    triggers: eisenmenger_confirmed
    Shunt closure is fatal; pregnancy carries ~30–50% maternal mortality; routine phlebotomy worsens iron deficiency/hyperviscosity (2020 ESC ACHD; 2022 ESC/ERS PH)

outpatient playbook — drug actions (3)

  1. 1. ERA first-line
    bosentan 62.5→125 mg BID (or macitentan/ambrisentan) • PO • BID/daily
    trigger: WHO FC II–IV Eisenmenger (BREATHE-5)
    Improves exercise/hemodynamics
  2. 2. add PDE5i ± prostacyclin pathway
    sildenafil 20 mg TID / selexipag uptitrated • PO • TID/BID
    trigger: Inadequate response / higher risk (2022 ESC/ERS PH)
    Goal-directed combination escalation
  3. 3. iron repletion (no routine phlebotomy)
    oral/IV iron per deficit • PO/IV • per protocol
    trigger: Iron deficiency (2018 AHA/ACC ACHD)
    Improves symptoms; avoid phlebotomy

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Central cyanosis / low SpO2 with congenital shunt + severe PAH; Secondary erythrocytosis (elevated Hb/Hct) with cyanotic CHD; Exertional dyspnea / syncope in shunt patient.

Objective

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

Assessment

**Eisenmenger syndrome (chronic ACHD — PAH-CHD severe end)** (cardio.achd-eisenmenger.chronic.v1).
Phenotype framing: Eisenmenger vs still-operable PAH-CHD (closable) vs other PAH/idiopathic
Scope: Confirm Eisenmenger physiology (severe PVR + R→L shunt); identify the underlying (non-closable) defect

No severity triggers fired against current inputs.

Plan

Regimen axis: **Eisenmenger — goal-directed PAH therapy + absolute-rule guardrails (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)** — step "Step 1 — Absolute-rule guardrails (every Eisenmenger patient)".
1. do NOT close the shunt; pregnancy CONTRAINDICATED (contraception counseling); avoid routine phlebotomy/dehydration/high-altitude/iatrogenic air emboli (safety_guardrail, first line) — Shunt closure is fatal; pregnancy carries ~30–50% maternal mortality; routine phlebotomy worsens iron deficiency/hyperviscosity (2020 ESC ACHD; 2022 ESC/ERS PH)

Setting playbook (outpatient) — Goal-directed PAH therapy, enforce absolute-rule guardrails (no closure/pregnancy/phlebotomy), iron repletion, multisystem surveillance, timely transplant (2018 AHA/ACC ACHD; 2022 ESC/ERS PH)
2. ERA first-line bosentan 62.5→125 mg BID (or macitentan/ambrisentan) PO BID/daily — WHO FC II–IV Eisenmenger (BREATHE-5) (Improves exercise/hemodynamics)
3. add PDE5i ± prostacyclin pathway sildenafil 20 mg TID / selexipag uptitrated PO TID/BID — Inadequate response / higher risk (2022 ESC/ERS PH) (Goal-directed combination escalation)
4. iron repletion (no routine phlebotomy) oral/IV iron per deficit PO/IV per protocol — Iron deficiency (2018 AHA/ACC ACHD) (Improves symptoms; avoid phlebotomy)

Non-pharmacologic actions:
- PAH-ACHD expert-centre management — 2022 ESC/ERS PH
- IE prophylaxis + IV air-filter / cerebral-abscess precautions — 2018 AHA/ACC ACHD
- Contraception counseling (pregnancy contraindicated) + non-cardiac-surgery precautions — 2018 AHA/ACC ACHD
- Transplant evaluation (heart-lung / lung+repair) for end-stage — 2020 ESC ACHD

AVOID / contraindication checks:
- Shunt closure CONTRAINDICATED in Eisenmenger fatal — 2020 ESC ACHD
- Pregnancy CONTRAINDICATED extreme maternal mortality contraception counseling — 2018 AHA/ACC ACHD
- No routine phlebotomy treat iron deficiency instead — 2018 AHA/ACC ACHD
- Anticoagulation individualised bleeding vs thrombosis not routine — 2020 ESC ACHD
- Non cardiac surgery very high risk expert centre only — 2018 AHA/ACC ACHD
- Meticulous IV air filters paradoxical embolism cerebral abscess — 2018 AHA/ACC ACHD

Monitoring

Regimen monitoring:
- WHO functional class and 6MWD — 2022 ESC/ERS PH
- RV function echo and NT-proBNP — 2022 ESC/ERS PH
- SpO2 and CBC iron studies — 2018 AHA/ACC ACHD
- PAH therapy response goal directed escalation — 2022 ESC/ERS PH
- renal uric acid gallstone surveillance — 2018 AHA/ACC ACHD

Setting (outpatient) monitoring:
- Functional class/6MWD/RV/NT-proBNP/SpO2/iron — 2022 ESC/ERS PH

Follow-up plan: Lifelong PAH-ACHD care; contraception + pregnancy-contraindication counseling; non-cardiac-surgery precautions
- Close-out criterion: lifelong plan + counseling documented

Monitoring phase: Functional class, RV function, SpO2, iron, PAH-therapy response

Disposition

Current setting: outpatient — Goal-directed PAH therapy, enforce absolute-rule guardrails (no closure/pregnancy/phlebotomy), iron repletion, multisystem surveillance, timely transplant (2018 AHA/ACC ACHD; 2022 ESC/ERS PH)

Disposition criteria:
- Confirmed Eisenmenger → PAH therapy + guardrails + expert-centre follow-up
- Still-operable PAH-CHD → route to defect engine for closure decision (NOT this engine)
- End-stage refractory → transplant evaluation

Escalation triggers (move to higher acuity):
- Massive hemoptysis / syncope / RV failure → emergency expert centre — 2022 ESC/ERS PH
- WHO FC III–IV / refractory → prostacyclin escalation + transplant evaluation — 2022 ESC/ERS PH
- Pregnancy → urgent expert counseling (termination discussion) — 2018 AHA/ACC ACHD

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Eisenmenger physiology (severe PVR + R→L shunt) — shunt closure is CONTRAINDICATED (fatal); do not refer for defect closure — 2020 ESC ACHD
- [LIFE_THREATENING] Pregnancy in Eisenmenger — CONTRAINDICATED (maternal mortality ~30–50%); urgent expert counseling, contraception, termination discussion — 2018 AHA/ACC ACHD
- [LIFE_THREATENING] Hemoptysis / pulmonary hemorrhage — potentially life-threatening; expert evaluation, reverse/avoid anticoagulation, imaging — 2022 ESC/ERS PH

Citations

- 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner); 2022 ESC/ERS PH + BREATHE-5 [PMID:30121239](https://pubmed.ncbi.nlm.nih.gov/30121239/)
- Cited evidence (PMID 32860028) [PMID:32860028](https://pubmed.ncbi.nlm.nih.gov/32860028/)
- Cited evidence (PMID 36017572) [PMID:36017572](https://pubmed.ncbi.nlm.nih.gov/36017572/)

Last reconciled with current guidelines: 2026-05-16.
References
  • 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner); 2022 ESC/ERS PH + BREATHE-5 — PMID:30121239
  • Cited evidence (PMID 32860028) — PMID:32860028
  • Cited evidence (PMID 36017572) — PMID:36017572