Skip to textbook content
Doctor’s PassportTextbook
TopicsAll topics

Loading topic navigation…

Textbook navigation
TopicsAll topics

Loading topic navigation…
Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Eisenmenger syndrome

Cardiology · UK scope · Sources checked 27 Aug 2026 · Clinical review pending

RapidFull
!
Escalate

Haemoptysis, syncope, chest pain, rapidly worsening cyanosis, neurological deficit, arrhythmia or right-heart failure requires immediate ABC care and urgent transfer advice from a combined ACHD-pulmonary-hypertension centre.

Synopsis

Recognise and safely manage pulmonary vascular disease with reversed congenital shunting, avoiding common iatrogenic harm.

  • Eisenmenger syndrome is severe pulmonary vascular disease caused by a longstanding systemic-to-pulmonary shunt, with bidirectional or right-to-left reversal and cyanosis.
  • Never close the causal shunt once Eisenmenger physiology is established.
  • Secondary erythrocytosis is an appropriate response to hypoxaemia; routine venesection is harmful.
  • Check iron status because iron deficiency worsens symptoms and makes microcytic blood less deformable despite a lower haemoglobin.
  • Avoid dehydration, systemic hypotension, air in intravenous lines and unplanned general anaesthesia.
  • Routine anticoagulation is not recommended without a separate indication because thrombosis and bleeding coexist.
  • Pregnancy carries very high maternal and fetal risk; provide expert pre-pregnancy counselling and Pregnancy Heart Team care if pregnancy occurs.

Key red flags

Acute decompensation

Syncope, rapidly falling saturation, arrhythmia, infection, haemoptysis or fluid overload is high risk.

Investigation priorities

01
Pulse oximetry at rest and with exertionFirst step

Document cyanosis and functional change.

Management branches

FirstNew suspected Eisenmenger physiology

Congenital shunt plus cyanosis or severe pulmonary hypertension.

  1. Do not arrange shunt closure; document anatomy, resting/exercise saturation and signs of right-heart failure.
  2. Check FBC with iron indices, renal/liver function, ECG and expert echo.
  3. Refer promptly to a combined ACHD-pulmonary-hypertension service for definitive classification and risk assessment.
  4. Give written advice on hydration, air filters for IV access, pregnancy, anaesthesia and emergency symptoms.
NextStable specialist management

Confirmed Eisenmenger syndrome without acute decompensation.

  1. Correct iron deficiency and other reversible contributors; avoid routine oxygen unless it produces clinical benefit or treats another indication.
  2. Use WHO functional class, exercise capacity, biomarkers and RV assessment to guide PAH-targeted therapy.
  3. For symptomatic patients, use an endothelin-receptor antagonist-based strategy and escalate combination therapy if response is inadequate.
  4. Plan dental care, vaccination, contraception, procedures and travel through the specialist service.

Key medicines

Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.Specialist initiation; teratogenic. Check liver transaminases before treatment and monthly and monitor haemoglobin. Bosentan reduces hormonal-contraceptive reliability: use effective additional or alternative contraception and perform monthly pregnancy testing.
Sildenafil20 mg orally three times daily for PAH.Never combine with nitrates or riociguat; consider hypotension, visual effects and CYP3A interactions. Specialist initiation and monitoring are required.
Open full textbook →Answer 2 questionsCardiology check
Sources and review status6 sources · checked 27 Aug 2026 · clinical review pending+
Sources

Sources and review status

National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.

  • ESC 2020 adult congenital heart disease guidelineEisenmenger diagnosis and management.
  • ESC/ERS 2022 pulmonary hypertension guidelineRisk assessment and PAH therapy.
  • ESC 2025 cardiovascular disease and pregnancy guidelinePregnancy risk and multidisciplinary care.
  • Bosentan 125 mg SmPCEisenmenger indication, dose and safety monitoring.
  • NHS England adult congenital heart servicesUK specialist service context.
  • Sildenafil PAH SmPCAdult PAH dose, interactions and contraindications.

Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom