Pulmonary hypertension - primary
Description
Haemodynamic definition (ESC/ERS 2022 - thresholds were lowered)
| Criterion | |
|---|---|
| Pulmonary hypertension | mPAP >20 mmHg at rest (was >=25) |
| Pre-capillary PH | mPAP >20 + PAWP <=15 + PVR >2 WU (was >3) |
| Isolated post-capillary | mPAP >20 + PAWP >15 + PVR <=2 |
| Combined pre- and post-capillary | mPAP >20 + PAWP >15 + PVR >2 |
| Exercise PH | mPAP/cardiac output slope >3 mmHg/L/min |
- *Requires right heart catheterisation. Echo estimates probability, it does not diagnose*
Clinical groups - the classification that decides treatment
| Group | Entity | Key point |
|---|---|---|
| 1 | Pulmonary arterial hypertension - idiopathic, heritable, drug/toxin-induced, CTD, HIV, portal hypertension, congenital heart disease, schistosomiasis | The only group with PAH-specific drug therapy |
| 1' | PVOD / PCH (pulmonary veno-occlusive disease) | *Vasodilators cause fulminant pulmonary oedema* |
| 2 | Left heart disease | Commonest group overall - treat the left heart |
| 3 | Lung disease / hypoxia - COPD, ILD, OSA, high altitude | Treat the lung; oxygen |
| 4 | CTEPH and other pulmonary artery obstruction | *Potentially curable by endarterectomy - never miss it* |
| 5 | Unclear/multifactorial - sarcoid, haematological (MPN, splenectomy), metabolic (Gaucher), CKD, fibrosing mediastinitis |
- *"Primary pulmonary hypertension" is an obsolete term - it is now idiopathic PAH (Group 1)*
- The single commonest error is treating Group 2 or 3 PH with PAH drugs - harmful, worsening V/Q matching and precipitating pulmonary oedema
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