Experience of Using the Non­selective Endothelin Receptor Antagonist Macitentan in Patients With Pulmonary Arterial Hypertension

Abstract

Aim. To present the results of treatment with macitentan of patients included in the clinical trial SERAPHIN in Almazov National Medical Research Centre and define target therapy of pulmonary arterial hypertension (PAH) in real clinical practice.Material and methods. The article presents the results of the 151 patients with PAH followed­up in Almazov National Medical Research Centre from 2009 to 2016.Results. Five­year survival of PAH patients included in the register of Almazov National Medical Research Centre reached 77% for idiopathic PAH, 52% for PAH associated with systemic scleroderma, 81% for PAH associated with non­repaired congenital heart disease, 82% for patients with repaired congenital shunts and 100% for PAH associated with human immunodeficiency virus infection. The group that received PAH specific monotherapy consisted of 47% (n=71) of patients among which phosphodiesterase type 5 inhibitor (PDE­5i) sildenafil was undoubtedly the most prescribed drug — 74% (n=53). The group that received combined PAH specific therapy consisted of 66 (44%) patients: 48 patients received various two­component therapy, 18 patients — threecomponent therapy with endothelin receptor antagonist in combination with PDE­5i and prostanoids. 11 patients with PAH were included in SERAPHIN study of which 9 patients had been already receiving generic PDE­5i therapy. In macitentan group, a statistically significant increase in the 6­minute walk distance (+50 meters) and a decrease in hemodynamic parameters such as mean right atrial pressure (­2,3 mm Hg) and pulmonary vascular resistance (­445 dyn­sec/cm5) were observed after 6 months. No patient had a clinically significant increase in liver transaminases or a decrease in hemoglobin levels.Conclusion. Improvement of prognosis in PAH patients according to the register of the Centre is connected both with early detection of the disease, thanks to the development of specialized healthcare, and more frequent use of combination therapy. Macitentan proved its long­term efficacy and safety as monotherapy and in combination with PDE­5i.

Principles of healthcare management for patients with pulmonary hypertension: focus on continuity

“…Среди всех нозологий, включенных в перечень хронических жизнеугрожающих и быстро прогрессирующих редких болезней, иЛАГ ассоциирована с крайне высокой смертностью и остается самой инвалидизирующей патологией [5][6][7]. Расчетная медиана выживаемости пациентов с иЛАГ в отсутствие лечения составляет 2,8 года [7].…”