This study will be performed based on a retrospective analysis of the cases of 150-170 patients with IPAH observed at the Research Institute of Clinical Cardiology over the past 10 years, for a comparative assessment of the survival of groups of patients with IPAH diagnosed in the period 2004-2013 and 2014-2023.

The demographic, clinical, functional, hemodynamic characteristics of patients, comorbidity, blood biomarker levels, received PAH-specific and symptomatic therapy will be assessed in order to determine the clinical characteristics of patients with IPAH.

The prospective part of study will include 50 patients with IPAH.

Inclusion Criteria:

• age over 18 years;
• verified diagnosis of IPAH

Exclusion Criteria:

• age under 18 years;
• Other etiology op PAH,
• ischemic heart disease confirmed by MSCT of the coronary arteries or coronary angiography;
• diseases of the musculoskeletal system that prevent 6MWT
• severe liver dysfunction (more than 9 points on the Child-Pugh scale, class C)
• severe renal dysfunction (CC less than 15 ml/min), need for hemodialysis

IPAH diagnosed patients in 2004-2023