Results of long-term riociguat therapy, including a switching strategy from sildenafil, in patients with pulmonary hypertension of various genesis

Aim: to study the influence of riociguat on the functional and hemodynamic status, remodeling of the right heart, as well as the safety of therapy in both previously untreated patients with idiopathic pulmonary arterial hypertension (IPAH) and inoperable chronic thromboembolic PH (CTEPH), and those not achieved treatment goals with sildenafil therapy and switching to riociguat.Materials and methods. A total of 161 pts with precapillary PH were included in the study; 137 pts completed the three-year observation period. Of 55 IPAH pts riociguat was started after diagnosis verification in 39 pts (subgroup 1); 16 pts previously taking sildenafil who did not achieve treatment goals comprised subgroup 2 of switching to riociguat. Of 82 inoperable CTEPH pts riociguat was started in 45 naїve pts; a switching strategy riociguat was implemented in 37 pts after 24-hour withdrawal of sildenafil. The dose titration of riociguat was started from 1 mg TID according to the standard algorithm up to 7.5 mg/day. By month 36 92.4% and 94.8% of pts with IPAH and CTEPH, respectively, received 7.5 mg/day. At baseline, at month 12, 24 and 36 all pts underwent a 6-minute walking test (6MWT) with the assessment of the dyspnea index according to the Borg scale and SpO2, echocardiography (Echo), right heart catheterization (RHC), and the safety profile was assessed.Results. At baseline, the proportion of pts with FC III-IV in CTEPH group compared to IPAH group, was significantly higher (70.7% vs 41.8%)); the distance in T6MX (d6MWT) was 291 [232;385] m vs 379 [300;448] m (p function show_eabstract() { $('#eabstract1').hide(); $('#eabstract2').show(); $('#eabstract_expand').hide(); }