The pandemic of a new coronavirus infection – COVID-19 («Coronavirus disease 2019») has changed the routine management of patients with pulmonary arterial hypertension (PAH). Like patients with other chronic cardiovascular diseases, PAH patients are not at higher risk of a new coronavirus infection. However, in the current epidemiological situation, the problems of managing high-risk groups are sharply indicated, in which the severe course of COVID-19 with a higher lethality is more often noted. Undoubtedly, рatients with different forms of pulmonary hypertension belong to the high-risk group, considering both the presence of severe lifethreatening cardiovascular pathology and background pathology in cases of the associated forms of the disease.At the same time, the first foreign experience indicates a rather low incidence and relatively favorable course of COVID-19 in PAH, which allows to formulate a number of hypotheses that will be confirmed or rejected in the near future. The unique problems of managing patients with PAH are those associated with changes in generally accepted standards of medical care and patient routing, which is especially important for patients receiving PAH-specific drugs. The problems of a complex assessment of the risk of possible infection in the event of hospitalization or an outpatient visit of patients on the one hand, and on the other, the risk of PAH progression with a delay in starting treatment, are a dilemma that requires an individual solution. Based on an analysis of current data, this review summarizes the main approaches to managing patients with PAH in the current epidemiological situation.

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Aim: evaluate the frequency of prescription and long-term efficacy of different types of specific therapy in patients with pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD).Materials and methods. The retrospective part of the study included patients first admitted to the National Medical Research Center of Cardiology in the period 2015-2017 — 30 patients with Eisenmenger syndrome, 25 with left-to-right shunt, 26 with residual PAH. 58 patients were followed up 12 and 24 months after the first hospitalization.Results. The most commonly prescribed drugs for PAH-CHD pts were sildenafil and bosentan as monotherapy and in combination. By the 24th month of followup there was a twofold increase of patients with combined specific therapy in all groups, the largest increase in patients with residual PAH. After 24 months, all modes of therapy showed positive dynamics with an increase in distance in the 6-minute walk testing, functional class improvement, a decrease in the mean pulmonary artery pressure, a decrease in the size of the right ventricle, and normalization of the left heart. The survival rate of patients with Eisenmenger syndrome was 80,5%, PAH-CHD with bidirectional shunt — 93,5%, residual PAH — 47,9%, idiopathic pulmonary hypertension — 48%.Conclusion. Patients with Eisenmenger syndrome receive specific monotherapy for a long time, while maintaining low/intermediate risk, are characterized by the best survival. Patients with residual PAH have the most severe hemodynamic impairment, rapid progression of the disease, despite the ongoing combination specific therapy, and low survival.

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