Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension. (March 2019)

• Record Type: Journal Article
• Authors: van Thor, M.C.J.; ten Klooster, L.; Snijder, R.J.; Post, M.C.; Mager, J.J.
• Is Part Of: IJC heart & vasculature. Volume 22(2019)
• Page Start: 163
• Page End: 168
• Publication Date: 2019-03
• Subjects: 6MWD 6-minute walking distance -- AE adverse event -- BPA balloon pulmonary angioplasty -- CO cardiac output -- CTEPH chronic thromboembolic pulmonary hypertension -- CHEST Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Trial -- CW clinical worsening -- e.g. exempli gratiā -- ERA endothelin receptor antagonist -- FC functional class -- HR hazards regression -- i.e. id est -- mPAP mean pulmonary arterial pressure -- NT-proBNP N-terminal pro brain natriuretic peptide -- PAH pulmonary arterial hypertension -- PAP pulmonary arterial pressure -- PEA pulmonary endarterectomy -- PH pulmonary hypertension -- PVR pulmonary vascular resistance -- RAP right atrial pressure -- Residual PH persistent pulmonary hypertension after PEA -- SD standard deviation -- sGC soluble guanylate cyclase -- WHO World Health Organization; Chronic thromboembolic pulmonary hypertension -- Riociguat -- Clinical outcome -- Survival -- Clinical worsening; Cardiovascular system -- Diseases -- Periodicals; Cardiovascular system -- Pathophysiology -- Periodicals; 616.1005
• DOI: 10.1016/j.ijcha.2019.02.004
• Languages: English
• ISSNs: 2352-9067

Abstract

Abstract: Background: To improve clinical outcome, patients with inoperable and residual chronic thromboembolic pulmonary hypertension (CTEPH) can be treated with riociguat. The aim of this study is to explore long-term outcomes and to compare our 'real world' data with previous research. Methods: We included all consecutive patients with technical inoperable and residual CTEPH, in whom riociguat therapy was initiated from January 2014 onwards, with patients followed till January 2019. Survival, clinical worsening (CW), functional class (FC), N-terminal pro brain natriuretic peptide (NT-proBNP) and 6-minute walking distance (6MWD) were described yearly after riociguat initiation. Results: Thirty-six patients (50% female, mean age 64.9 ± 12.1 years, 54% WHO FC III/IV and 6MWD 337 ± 138 m could be included, with a mean follow-up of 2.3 ± 1.2 years. Survival and CW-free survival three years after initiation of riociguat were 94% and 78%, respectively. The 6MWD per 10 m at baseline was a significant predictor (HR 0.90 [0.83–0.97], p = 0.009) for CW. At three years follow-up the WHO FC and 6MWD improved and NT-proBNP decreased compared to baseline. Conclusion: Our study confirms that riociguat is an effective treatment in patients with technical inoperable and residual CTEPH at long-term follow-up. Although our results are consistent with previous studies, more 'real world' research is necessary to confirm long-term results.