Pulmonary arterial hypertension: regional health care opportunities

Over the past few decades, there have been significant changes in approaches to the management of patients with pulmonary arterial hypertension (PAH). From the moment when the classification of this disease was first published, new diagnostic capabilities and new drugs have appeared that have improved the quality of life and survival of patients. The article is devoted to the issues of rapid diagnosis of PAH in the case of its high probability. Particular attention is paid to the rapid referral of such patients to an expert center, where it is possible to perform catheterization of the right heart. The importance of risk stratification of patients is also noted, which allows prescribing combination specific therapy at an early stage, which can significantly improve the quality of life and reduce the risk of adverse outcomes. Risk assessment is indicated for all patients at both regional and expert level by determining the risk of 1-year mortality in PAH. The article also discusses the issues of dispensary observation of such patients and dose titration when prescribing specific therapy by an expert center. Recommendations are given for non-specific therapy for PAH, the use of oxygen support, anticoagulants, and other drugs. It is emphasized that ACE inhibitors, angiotensin receptor blockers, betablockers, and ivabradine are generally not used to treat PAH, and their use may limit the possibilities of specific therapy. Occasionally, patients with PH at the regional level require dose titration of PAH-specific therapy, as well as regular follow-up, which requires specialized knowledge. This dictates the need to create pulmonary hypertension rooms in each region or manage this group of patients in high-risk rooms, where medical workers will have the necessary knowledge and will regularly undergo training in expert centers. The described clinical case demonstrates the effectiveness of combination therapy of pulmonary arterial hypertension using an innovative selective agonist of prostacyclin receptors for oral administration - Selexipag. Practical recommendations are given for dose titration and its use in patients with PAH.