Pulmonary arterial hypertension therapy reference (2026)
Reference snapshot of PAH therapy across the four established mechanism pathways and emerging activin-pathway tier.
PAH therapy in 2026 organises around four established pathways and a fifth emerging class.
Endothelin receptor antagonists: bosentan, ambrisentan, macitentan.
PDE5 inhibitors and soluble guanylate cyclase stimulators: sildenafil, tadalafil, riociguat.
Prostacyclin pathway agents: epoprostenol IV, treprostinil (IV, sub-cutaneous, inhaled, oral), iloprost, selexipag (oral prostacyclin agonist), with oral prostacyclin programs in late-stage trials.
Activin-pathway: sotatercept, with follow-on activin-pathway programs in trials.
Upfront combination therapy is the modern standard for newly-diagnosed PAH; the specific combination depends on risk stratification. Lung transplant remains an option for end-stage disease. Diagnostic delay continues to be a major issue; symptoms are often attributed to deconditioning or other causes for a year or more before correct identification.
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