Disease-targeted therapies for PAH have been shown to improve survival or prognostic indicators and there are studies demonstrating this for prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors and soluble guanylate cyclase stimulators. These are summarised in the 2015 guidelines.
Baseline prognostic indicators in PAH include functional class, exercise capacity, right ventricular function and NTproBNP. For more details, see the ESC guidelines on this link.