Pulmonary hypertension
Haemodynamic definition, the five clinical groups, the work-up that finds CTEPH and PAH, risk stratification and PAH drug therapy.
Tier 1Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Definition: mean pulmonary artery pressure (mPAP) over 20 mmHg at rest on right heart catheterisation (RHC). Echo estimates suspicion; only RHC diagnoses.
- Left heart disease is the commonest cause, then lung disease. PAH (group 1) is rare but has specific, effective drugs.
- Everyone with unexplained PH needs a V/Q scan to look for CTEPH, which is potentially curable.
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Sources
- Humbert M, Kovacs G, Hoeper MM et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022;43:3618-3731. doi:10.1093/eurheartj/ehac237European Society of Cardiology and European Respiratory Society, 2022
- Kovacs G, Bartolome S, Denton CP et al. Definition, classification and diagnosis of pulmonary hypertension (7th World Symposium on Pulmonary Hypertension). Eur Respir J 2024;64:2401324European Respiratory Society, 2024
- Kovacs G, Zeder K, Humbert M et al. European Respiratory Society clinical practice guidelines update for the treatment of pulmonary arterial hypertension. Eur Respir J 2026 (accepted manuscript)European Respiratory Society, 2026
- Hoeper MM, Badesch DB, Ghofrani HA et al. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension (STELLAR). N Engl J Med 2023;388:1478-90New England Journal of Medicine, 2023
- Humbert M, McLaughlin VV, Badesch DB et al. Sotatercept in patients with pulmonary arterial hypertension at high risk of death (ZENITH). N Engl J Med 2025;392:1987-2000New England Journal of Medicine, 2025
- Last updated
- 14 November 2026
What changed
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