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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.

Pulmonary hypertension

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

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Escalate

Syncope, hypotension, rising lactate, severe hypoxaemia, new arrhythmia or right-heart failure in pulmonary hypertension is a high-mortality emergency; avoid large unassessed fluid boluses and seek immediate PH-centre/critical-care advice.

Synopsis

Recognise pulmonary hypertension early, classify its cause correctly and refer suspected PAH or CTEPH to nationally commissioned specialist care.

  • Pulmonary hypertension is haemodynamically defined by mean pulmonary artery pressure greater than 20 mmHg at rest on right-heart catheterisation.
  • Pre-capillary PH also requires pulmonary artery wedge pressure 15 mmHg or less and pulmonary vascular resistance greater than 2 Wood units.
  • Echo estimates probability; it does not confirm PH or distinguish all five clinical groups.
  • Use a three-step approach: clinical suspicion, echocardiographic detection, then confirmation/classification in a PH centre.
  • Always consider left-heart disease, lung/hypoxic disease and chronic thromboembolic PH before labelling PAH.
  • A ventilation-perfusion scan is the key screening test for CTEPH; a normal perfusion scan makes it unlikely.
  • PAH-targeted drugs should be initiated and escalated by a specialist PH service, generally using risk-based combination therapy.

Key red flags

High-risk presentation

Exertional syncope, hypotension, WHO functional class IV symptoms or rapidly worsening right-heart failure.

Investigation priorities

01
ECG, chest radiograph and basic blood testsFirst step

Look for RV strain, alternative disease, anaemia, renal/liver dysfunction, thyroid disease and treatment constraints.

Management branches

FirstClinical suspicion

Unexplained dyspnoea, syncope, RV signs or a condition associated with PAH/CTEPH.

  1. Assess acuity, oxygen saturation, ECG, chest radiograph, FBC, renal/liver/thyroid tests and BNP/NT-proBNP.
  2. Obtain expert echocardiography to grade PH probability and examine left heart, RV and shunts.
  3. Investigate common causes with lung testing/imaging and a V/Q scan rather than assuming idiopathic PAH.
  4. Refer intermediate/high echo probability, suspected PAH or CTEPH to an NHS specialist PH centre.

Key medicines

Sildenafil20 mg orally three times daily for PAH.Contraindicated with nitrates and riociguat; hypotension, visual effects and CYP3A interactions.
Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.Teratogenic; monthly liver tests, haemoglobin monitoring and major drug interactions including reduced hormonal-contraceptive reliability.
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Sources and review status5 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/ERS 2022 pulmonary hypertension guidelineDefinition, diagnosis, classification and treatment.
  • European Respiratory Journal full guidelineOfficial joint ESC/ERS guideline text.
  • NHS England specialised respiratory servicesAdult PH centres and commissioned services.
  • Riociguat SmPCCTEPH/PAH dose, titration and contraindications.
  • Sildenafil PAH SmPCAdult PAH dosing and safety.

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