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.
Hepatopulmonary syndrome and portopulmonary hypertension
Essential points for quick revision.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
!
Escalate
Syncope, resting hypoxaemia, rapidly increasing oxygen requirement, chest pain, haemoptysis or signs of right-heart failure require urgent assessment. Give oxygen for significant hypoxaemia, evaluate common acute cardiopulmonary causes and contact hepatology, respiratory or the designated pulmonary-hypertension centre early. Severe uncontrolled portopulmonary hypertension can make liver transplantation immediately hazardous, whereas severe hepatopulmonary syndrome may increase its urgency; neither diagnosis should be inferred from breathlessness alone.
Synopsis
Distinguish two pulmonary vascular complications of portal hypertension, confirm each with the right test, and coordinate cardiopulmonary optimisation with liver-transplant decisions.
Hepatopulmonary syndrome combines liver disease or portal hypertension, intrapulmonary vascular dilatation and an objectively demonstrated oxygenation defect.
Platypnoea and orthodeoxia favour hepatopulmonary syndrome because upright posture increases blood flow through basal dilated pulmonary vessels.
Agitated-saline contrast appearing in the left heart after a delayed passage supports intrapulmonary shunting; immediate passage suggests an intracardiac communication.
Key red flags
Pulmonary hypertension symptoms
Exertional dyspnoea, chest pressure, presyncope or syncope, ankle swelling and declining exercise capacity suggest right-ventricular stress and require specialist investigation.
Investigation priorities
01
Pulse oximetry supine and uprightFirst step
Screen for hypoxaemia and document a clinically relevant postural change.
Management branches
HPSConfirm oxygenation and shunt
A person with liver disease or portal hypertension has unexplained dyspnoea, low saturation or orthodeoxia.
Measure saturation with posture, obtain a properly documented room-air arterial blood gas where safe, and investigate common lung, cardiac, anaemic and thromboembolic explanations in parallel.
Arrange agitated-saline contrast echocardiography through an experienced service, interpreting delayed left-sided bubbles as evidence of intrapulmonary vascular passage in the correct clinical context.
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.