Abstract
PAH associated with CHD according to the Russian Register of Pulmonary Arterial Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is highly prevalent and ranks second after idiopathic pulmonary hypertension (IPH) in terms of prevalence among all forms of PAH and the first among associated forms. With the development of PAH, patients with congenital heart disease (CHD) require the appointment of a constant PAH-specific therapy. The article presents an overview of clinical trials of medications currently registered in the Russian Federation for this cohort of patients, including endothelin receptor antagonists (bosentan, macitentan, ambrisentan), phosphodiesterase type 5 inhibitors (sildenafil), soluble guanylate cyclase stimulants (riociguat) and prostacyclin analogues (iloprost).
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