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pulmonary-arterial-hypertension
Pulmonary Arterial Hypertension
PAH (WHO Group 1) is progressive obliterative pulmonary vascular disease; mPAP >20 mmHg + PVR ≥2 WU; BMPR2/BMP9 mutations in heritable PAH. Three pathways (endothelin/NO/prostacyclin) targeted by ERAs, PDE5i/sGCi, and prostacyclin analogues.
Entry Metadata
| Field | Value |
|---|---|
| ID | pulmonary-arterial-hypertension |
| Name | Pulmonary Arterial Hypertension |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67-119. · PubMed 26320113
- Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. · PubMed 30545968
- Sitbon O, Channick R, Chin KM, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015;373(26):2522-2533. · PubMed 26579977