Treatment of pulmonary arterial hypertension: current questions

• Klimenlo Alesya A.
• Shostak Nadezhda A.
• Andriyashkina Darya Yu.
• Kondrashov Artem A.

Abstract

Pulmonary hypertension (PH) is a group of potential and life-threatening diseases characterized by a progressive increase of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) with the rapid outcome as the right ventricular failure. The treatment of patients with pulmonary arterial hypertension (PAH) consists of some essential steps: general measures; initial PAH-specific therapy; combined PAH-specific therapy and interventional methods (in case of inadequate response to the targeted therapy). This article aims to demonstrate the possibilities of targeted therapy for PAH in adults with the known four classes of drugs: endothelin receptor antagonist, prostacyclin analogues, phosphodiesterase type 5 inhibitor (PDE5 inhibitor), soluble guanylate cyclase (sGC stimulator) based on updated of the CHEST guideline and Expert panel report (The American College of Chest Physicians - ACCP or CHEST) published in 2019. The 6-minute walking distance was chosen as a clinically relevant outcome, which allowed ACCP experts to extract data from clinical studies AMBITION and GRIPHON. Great attention is paid to the initial combined therapy for patients with PAH, the results of a series of randomized controlled trials of the combination of ambrisentan and tadalafil are discussed, the interim results of a prospective open-label study of the initial combination of ambrisentan and riociguat are demonstrated. In addition, contemporary data from updated recommendations from the Cologne Consensus Conference 2018 are also presented, which testify in favor to initial combination therapy for naive young patients with PAH without significant cardiopulmonary comorbidities and abstinence from it for elderly patients with significant cardiopulmonary comorbidities. As part of the updated recommendations, it is also possible to change PAH-specific therapy from PDE5 inhibitor to sGC stimulators on an individual basis, which in some cases may be the best approach in comparison with escalation of therapy.

Keywords:pulmonary hypertension, pulmonary artery hypertension, PAH-specific therapy, soluble guanylate cyclase stimulator, riociguat, phosphodiesterase type 5 inhibitor, endothelin receptor antagonist, initial combination therapy

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