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Lookup NU author(s): Emeritus Professor Nick Europe-Finner
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© ERS 2017. A proportion of pulmonary arterial hypertension (PAH) patients do not reach treatment goals with phosphodiesterase-5 inhibitors (PDE5i). RESPITE investigated the safety, feasibility and benefit of switching from PDE5i to riociguat in these patients. RESPITE was a 24-week, open-label, multicentre, uncontrolled study. Patients in World Health Organization (WHO) functional class (FC) III, with 6-min walking distance (6MWD) 165-440 m, cardiac index <3.0 L min-1 m-2 and pulmonary vascular resistance >400 dyn s cm-5 underwent a 1-3 day PDE5i treatmentfree period before receiving riociguat adjusted up to 2.5 mg maximum t.i.d. Exploratory end-points included change in 6MWD, WHO FC, N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and safety. Of 61 patients enrolled, 51 (84%) completed RESPITE. 50 (82%) were receiving concomitant endothelin receptor antagonists. At week 24, mean±SD 6MWD had increased by 31±63 m, NT-proBNP decreased by 347±1235 pg mL-1 and WHO FC improved in 28 patients (54%). 32 patients (52%) experienced study drug-related adverse events and 10 (16%) experienced serious adverse events (2 (3%) study drug-related, none during the PDE5i treatment-free period). Six patients (10%) experienced clinical worsening, including death in two (not study drug-related). In conclusion, selected patients with PAH may benefit from switching from PDE5i to riociguat, but this strategy needs to be further studied.
Author(s): Hoeper MM, Simonneau G, Corris PA, Ghofrani H-A, Klinger JR, Langleben D, Naeije R, Jansa P, Rosenkranz S, Scelsi L, Grunig E, Vizza CD, Chang M, Colorado P, Meier C, Busse D, Benza RL
Publication type: Article
Publication status: Published
Journal: European Respiratory Journal
Year: 2017
Volume: 50
Issue: 3
Online publication date: 09/09/2017
Acceptance date: 12/07/2017
ISSN (print): 0903-1936
ISSN (electronic): 1399-3003
Publisher: European Respiratory Society
URL: https://doi.org/10.1183/13993003.02425-2016
DOI: 10.1183/13993003.02425-2016
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