Riociguat for pulmonary hypertension – experience from a referral center

Background: Several achievements have been made in the treatment of pulmonary hypertension (PH). Soluble guanylate cyclase (sGC) stimulator Riociguat has been approved for the treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) and is available in Europe since April 2014. Methods: A one-year prospective data collection of PH patients that started oral targeted therapy after right heart catheterization (RHC) at our center from April 2014. Parameters of treatment efficacy were prospectively assessed. Results: 56 PAH and 49 CTEPH patients had a RHC before initiation of targeted oral therapy. Baseline characteristics of the patients showed severely impaired hemodynamics and exercise capacity, as shown in table 1. 24 patients started phosphodiesterase type 5 inhibitor (PDE5i), 29 patients started endothelin receptor antagonists (ERA), and 52 patients sGC-stimulator treatment. While 42 of the 49 CTEPH patients were started on Riociguat, treatment in PAH was different: 21 PDE5i, 25 ERA, and 10 Riociguat. While Riociguat and PDE5i were first line treatment in over 70%, this was the case only in 7% of the ERA starters. Tab. 1: pulmonary hemodynamics and six-minute-walk distance by etiologic class. PAH CTEPH PAP [mmHg] 46 ± 11 41 ± 10 RAP [mmHg] 6 ± 5 7 ± 5 PAWP [mmHg] 8 ± 3 10 ± 5 CI [l/min/m2] 2,4 ± 0,7 2,3 ± 0,6 PVR [dyne] 779 ± 449 666 ± 292 6MWD [m] 342 ± 162 330 ± 145 Conclusion: Prospective collection of real life prescription habits in a large PH referral center since approval of first in class sGC stimulator.