Experience of idiopathic pulmonary arterial hypertension patients management in Ural federal district: features of outpatient-hospital interactions

Idiopathic pulmonary arterial hypertension (IPAH) is a severe chronic and rapidly progressive disease with increasing pulmonary vascular resistance (PVR) due to the ongoing pathogenic processes in the vascular wall that lead to obstruction of small pulmonary arteries and arterioles. IPAH takes a small part in the structure among all forms of pulmonary hypertension (PH), the median survival of patients with IPAH before any PAH-specific therapy became available were about 2,8 years, and the average time from the manifestation of the disease to its diagnosis took 2-3 years. This article is an attempt to present an unified data of adult patients with IPAH in Ural Federal district, and describe PH patients medical care service. The article presents the experience of PH Medical Centers across Ural Federal District.