Patient handout

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

PRODUCTION

1. Your condition

This handout is for eisenmenger syndrome (chronic achd — pah-chd severe end). Your care team identified this based on: central cyanosis / low spo2 with congenital shunt + severe pah.

Other reasons your team may use this plan: secondary erythrocytosis (elevated hb/hct) with cyanotic chd; exertional dyspnea / syncope in shunt patient; hemoptysis (pulmonary hemorrhage risk).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

Medication	Starting dose	How	When	What it does
do NOT close the shunt; pregnancy CONTRAINDICATED (contraception counseling); avoid routine phlebotomy/dehydration/high-altitude/iatrogenic air emboli	—	—	—	Shunt closure is fatal; pregnancy carries ~30–50% maternal mortality; routine phlebotomy worsens iron deficiency/hyperviscosity (2020 ESC ACHD; 2022 ESC/ERS PH)

Plan: Eisenmenger — goal-directed PAH therapy + absolute-rule guardrails (2018 AHA/ACC ACHD; 2020 ESC ACHD; 2022 ESC/ERS PH)

3. When to call your provider

Contact your care team if any of the following happen:

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

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

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(life-threatening)
Any non-cardiac surgery / anesthesia — very high perioperative mortality; expert-centre management, avoid systemic vasodilation/hypovolemia, air-filter precautions — 2018 AHA/ACC ACHD(life-threatening)
Right-to-left shunt → paradoxical embolism / cerebral abscess risk — meticulous IV air filters; fever/neuro symptoms → urgent imaging — 2018 AHA/ACC ACHD
Atrial/ventricular arrhythmia — poorly tolerated in Eisenmenger; expert rhythm management (avoid pro-arrhythmic destabilisation) — 2020 ESC ACHD
Refractory WHO FC III–IV despite goal-directed therapy — heart-lung or lung+repair transplant evaluation — 2020 ESC ACHD(life-threatening)

5. Follow-up

Lifelong PAH-ACHD care; contraception + pregnancy-contraindication counseling; non-cardiac-surgery precautions

6. Sources

Guideline: 2018 AHA/ACC Adult Congenital Heart Disease Guideline (Stout) + 2020 ESC Adult Congenital Heart Disease Guideline (Baumgartner); 2022 ESC/ERS PH + BREATHE-5