Pulmonary hypertension
PH = mPAP ≥20 mmHg. Precapillary: PVR >2 and PCWP ≤15. Postcapillary: PCWP >15, PVR ≤2. Mixed: both high.
Dan Rubin & Rahul Gupta5 min6 questions3 trials
Define it, then group it
Pulmonary hypertension is mPAP >20 mmHg. Precapillary (PAH and most of groups 3/4): PVR >2 WU and PCWP ≤15. Isolated post-capillary is left-heart disease ().
| Group | Mechanism | Do not |
|---|---|---|
| 1 PAH | Pulmonary arterial remodeling — idiopathic, heritable, CTD, portal, HIV, drugs | Stop epoprostenol on admission |
| 2 | Left heart (HFpEF, HFrEF, left valvular) | Start PAH meds without a RHC that proves precapillary disease |
| 3 | Lung disease / hypoxia | Treat the lung and oxygen first |
| 4 CTEPH | Chronic clot | Miss the V/Q. This one is potentially operable. |
| 5 | Multifactorial (sarcoid, sickle, ESRD, myeloproliferative) | Assume one pathway |
Risk
Stratify
WHO FC, 6MWT, NT-proBNP, echo, hemodynamics. High-risk gets a parenteral prostacyclin.
Dual oral
AMBITION
Upfront ERA + PDE5i (ambrisentan + tadalafil) beat sequential monotherapy ().
Add-on
Sotatercept
Activin-pathway inhibitor on background therapy (STELLAR, NEJM 2023). Watch hemoglobin and telangiectasias.