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.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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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.
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.
Do not arrange shunt closure; document anatomy, resting/exercise saturation and signs of right-heart failure.
Check FBC with iron indices, renal/liver function, ECG and expert echo.
NextStable specialist management
Confirmed Eisenmenger syndrome without acute decompensation.
Key medicines
Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.
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.