TY - JOUR T1 - Long-term riociguat treatment towards implementing a targeted treatment strategy in patients with idiopathic pulmonary arterial hypertension JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2020.1465 VL - 56 IS - suppl 64 SP - 1465 AU - Anna Aleevskaya AU - Svetlana Gratsianskaya AU - Zarina Valieva AU - Tamila Martynyuk Y1 - 2020/09/07 UR - http://erj.ersjournals.com/content/56/suppl_64/1465.abstract N2 - Aim: To evaluate long-term efficacy of riociguat treatment in pts with idiopathic pulmonary arterial hypertension (IPAH) for 24 months.Methods: 28 pts with IPAH (WHO functional class (FC) II/III 36%/64%) were included in the prospective observation study as part of the Russian National Registry (NCT03707561). The mean time from symptom onset to riociguat therapy was 34,9 ± 44,2 months. Аt Month 13, iloprost (n=4) and bosentan (n=5) were added to riociguat, 19 pts (68%) achieved low risk and continued riociguat monotherapy.Results: The mean age of pts was 39,2±10,7 years. 16 pts (57%) had not previously received PAH-targeted therapy. 12 pts (43%) received sildenafil (60-80 mg) but failed to achieve treatment goals. These 12 pts were switched from sildenafil to riociguat. The median from start of PAH-targeted therapy to transition to riociguat was 13,5[7,5; 18,7] months. During 6 months from the initiation of riociguat therapy 10 pts (36%) were not taking riociguat and 6 pts (29%) were not completing of titration daily dose (receive <3 mg) due to problems of drug reimbursement. At Month 24, riociguat improved WHO FC (from 0/10/18/0 pts in WHO FC I/II/III/IV at baseline to 11/9/8/0 at Month 24) and increased 6-minute walking distance by 104,2 m from baseline. Treatment goals were achieved by Month 6 and continued for 24 months (from 6/15/7 pts in low/intermediate/high risk at baseline to 19/9/0 at Month 12 and 19/9/0 at Month 24). The 24-month survival rate was 100%.Conclusion: In IPAH pts with WHO FC II-III, riociguat proved a long-term effect. By 13 months escalation therapy was required in 32% of pts. The 24-month survival rate was 100%.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 1465.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -