Abstract
Advancements in the specific therapy of pulmonary arterial hypertension (PAH) and new combination therapy strategies and escalation treatment over the past decade have significantly improved disease control, slowed its progression, and improved quality of life and patient survival. This review provides a detailed discussion of current strategies for PAH-specific therapy available in the Russian Federation for patients with classical and non-classical PAH who fail to achieve treatment goals and remain at intermediate risk of disease progression/mortality.