How is the general treatment approach in PH
Treat acute decompensated PH urgently, if present.
Determine functional severity and screen for high-risk features; consult PAH specialist and strongly consider admission to PAH specialty center if present.
Tailor treatment according to WHO PH group
Start supportive treatment to reduce symptoms and slow progression.
Consider directed therapies for Group I PH (i.e., PAH): e.g., CCBs and/or other pulmonary vasodilators.
Treat the underlying cause of PH (e.g., CPAP for patients with OSA, CHF treatment, anticoagulation for PE).
How to assess severity?
WHO-FC I: No limitations in physical activity; no significant symptoms during most regular ADLs and IADLs
WHO-FC II: Slightly limited physical activity; no rest symptoms, but some symptoms during IADLs
WHO-FC III: Noticeably limited physical activity; consistent symptoms during basic ADLs
WHO-FC IV: Severely limited physical activity; frequent and/or progressive symptoms with almost any activity or even at rest
Name high-risk features
The presence of any of the following features typically warrants admission to a PAH specialty center for evaluation and treatment. Stabilization in an ICU setting may be required in some cases.
Rapidly progressing symptoms
Unclear PH etiology
Severe PH (e.g., WHO-FC III-IV)
Significantly elevated NT-proBNP