PassFinals
Cardiovascular

Pulmonary hypertension

Pulmonary hypertension is a haemodynamic disorder in which the pulmonary circulation places an abnormal load on the right ventricle; diagnosis requires specialist classification because treatment differs between pulmonary arterial, left-heart, lung-disease, chronic-thromboembolic and multifactorial disease.

In a nutshell

Pulmonary hypertension is confirmed by right-heart catheterisation, not echocardiography alone. Classify it into pulmonary arterial, left-heart, lung or hypoxic, chronic thromboembolic, or multifactorial disease because treatment differs. Always exclude chronic thromboembolic disease with V/Q assessment, refer suspected significant disease to a specialist centre, and use targeted vasodilators only within the relevant specialist pathway.

Classic presentation

A patient with progressive exertional breathlessness, presyncope or syncope has a loud P2, right-ventricular heave and raised JVP; echocardiography suggests pulmonary hypertension, and specialist right-heart catheterisation confirms and classifies the haemodynamic pattern.

Key points

  • The haemodynamic definition is mean pulmonary arterial pressure above 20 mmHg at rest; pre-capillary disease also requires wedge pressure 15 mmHg or less and pulmonary vascular resistance above 2 Wood units.
  • Echocardiography estimates probability and mechanism; right-heart catheterisation confirms the diagnosis and classifies pre-capillary versus post-capillary disease.
  • Think in five clinical groups: PAH, left-heart disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear disease.
  • A V/Q scan is the key screening test for chronic thromboembolic disease and should not be replaced by a negative or non-diagnostic CTPA alone.
  • Exertional syncope, right-heart failure, hypotension, hypoxaemia or rapid progression are high-risk features needing urgent specialist escalation.
  • Do not use PAH-targeted vasodilators indiscriminately in group 2 or group 3 disease; treat the underlying heart or lung disease through the appropriate pathway.
  • NICE TA1161 supports sotatercept with other PAH treatment in selected adults with WHO functional class 2 or 3 PAH when started at intermediate-low risk at follow-up after usual treatment.
  • Chronic thromboembolic pulmonary hypertension requires specialist operability assessment because pulmonary endarterectomy may be curative and balloon angioplasty is a specialist alternative.

First-line investigation

Transthoracic echocardiography to estimate probability and assess right- and left-heart structure, followed by specialist right-heart catheterisation and V/Q assessment when pulmonary hypertension is likely.

Management

Recognise high-risk right-heart failure

  • Escalate exertional syncope, hypotension, severe hypoxaemia, rapidly worsening symptoms, acute right-heart failure or low cardiac output to specialist and critical-care teams; pregnancy in known PAH needs urgent specialist planning.1,7,4

Screen and classify rather than anchor

  • Use echocardiography to estimate probability, then confirm with specialist right-heart catheterisation and complete V/Q assessment and targeted aetiology testing before prescribing targeted therapy.1,2,7

Treat the underlying group and support the right ventricle

  • Optimise left-heart or lung disease, correct hypoxaemia where indicated, treat congestion carefully, provide rehabilitation and vaccination advice, and avoid unindicated PAH drugs in group 2 or group 3 disease.1,3,8,9

Use specialist targeted or surgical treatment

  • Use risk-based PAH combination therapy and selected NICE TA1161 sotatercept through the specialist centre; refer chronic thromboembolic disease for endarterectomy, balloon angioplasty or specialist medical treatment according to operability and residual risk.1,6,5,9

Review risk, treatment toxicity and advanced options

  • Reassess functional status, exercise capacity, symptoms, biomarkers, right-ventricular function, oxygen needs and treatment adverse effects; escalate persistent high risk to advanced therapies or transplant discussion through the pulmonary hypertension MDT.1,6,7

Exam traps

  • Pulmonary artery pressure estimated on echocardiography is not the same as a confirmed haemodynamic diagnosis.
  • The current threshold is mean pulmonary arterial pressure above 20 mmHg, but PAH also requires a pre-capillary pattern with wedge pressure and pulmonary vascular resistance criteria.
  • A normal or non-diagnostic CTPA does not replace V/Q scanning when chronic thromboembolic disease is suspected.
  • Group 2 and group 3 pulmonary hypertension is not managed by automatically prescribing group 1 PAH vasodilators.
  • Right-ventricular function and clinical risk, not the pressure number alone, determine urgency and prognosis.
  • NICE TA1161 is a selected PAH recommendation and does not apply to undifferentiated pulmonary hypertension or other clinical groups.

Illustrations

Cardiomegaly and central pulmonary artery enlargementA chest radiograph showing cardiomegaly and prominent central pulmonary arterial contours in advanced pulmonary hypertension, with a legend warning that radiography supports but cannot confirm the diagnosis.Ishida N et al., Journal of Cardiothoracic Surgery, CC-BY-4.0 · CC-BY-4.0

Key sources

  1. ESC/ERS: 2022 Guidelines for the diagnosis and treatment of pulmonary hypertension (Comprehensive current international guideline covering all clinical groups, haemodynamic definitions, diagnosis, risk assessment and treatment; published 26 August 2022 and accessed 4 August 2026. Used because there is no single comprehensive UK clinical guideline.)Updated 26 Aug 2022
  2. NHS: Pulmonary hypertension—diagnosis (NHS diagnostic overview including echocardiography, right-heart catheterisation, lung function tests, V/Q scanning and specialist-centre confirmation; page last reviewed 2 May 2023 and accessed 4 August 2026.)Updated 2 May 2023
  3. NICE NG115: Chronic obstructive pulmonary disease in over 16s: diagnosis and management (NICE COPD pathway, including the warning not to use pulmonary vasodilators solely to manage pulmonary hypertension caused by COPD outside a randomised controlled trial; current recommendations accessed 4 August 2026.)
  4. NHS: Pulmonary hypertension (UK NHS overview of clinical groups, symptoms, complications and treatment; page last reviewed 2 May 2023 and accessed 4 August 2026.)Updated 2 May 2023
  5. NICE HTG410: Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension (NICE specialist governance, selection and treatment recommendations for balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension; published 27 April 2016, current guidance accessed 4 August 2026.)
  6. NICE TA1161: Sotatercept for treating pulmonary arterial hypertension (Current NICE option with other PAH treatments for selected adults with WHO functional class 2 or 3 PAH, started at intermediate-low risk at follow-up after usual treatment and continued if progression to intermediate-high risk; published and last reviewed 3 June 2026, accessed 4 August 2026.)Updated 3 Jun 2026
  7. NHS England: Specialised service specifications—pulmonary hypertension centres and shared care (Current NHS England specialised-commissioning service-specification library listing adult pulmonary hypertension centres, shared care and pulmonary thromboendarterectomy services; accessed 4 August 2026.)
  8. NHS: Pulmonary hypertension—treatment (NHS overview of underlying-cause treatment, anticoagulation, diuretics, pulmonary vasodilators and oxygen; page accessed 4 August 2026.)
  9. British National Formulary (BNF) (BNF online prescribing, contraindication, interaction, monitoring and specialist-use information for pulmonary hypertension therapies, anticoagulants, diuretics and oxygen-related medicines; accessed 4 August 2026.)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.