TY - JOUR
T1 - Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension
AU - van Thor, M. C.J.
AU - ten Klooster, L.
AU - Snijder, R. J.
AU - Post, M. C.
AU - Mager, J. J.
N1 - Funding Information:
M. van Thor reports grants from Actelion Pharmaceuticals, outside the submitted work. L ten Klooster has nothing to disclose. R. Snijder reports grants from Pfizer and Actelion Pharmaceuticals, outside the submitted work. M. Post reports grants and speaking fees from Actelion Pharmaceuticals and grants from GlaxoSmithKline, outside the submitted work. J.J. Mager reports grants from Actelion Pharmaceuticals, outside the submitted work.
Publisher Copyright:
© 2019 The Authors
Copyright:
Copyright 2019 Elsevier B.V., All rights reserved.
PY - 2019/3/1
Y1 - 2019/3/1
N2 - 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.
AB - 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.
KW - Chronic thromboembolic pulmonary hypertension
KW - Clinical outcome
KW - Clinical worsening
KW - Riociguat
KW - Survival
UR - http://www.scopus.com/inward/record.url?scp=85062031183&partnerID=8YFLogxK
U2 - 10.1016/j.ijcha.2019.02.004
DO - 10.1016/j.ijcha.2019.02.004
M3 - Article
C2 - 30859124
AN - SCOPUS:85062031183
SN - 2352-9067
VL - 22
SP - 163
EP - 168
JO - IJC Heart and Vasculature
JF - IJC Heart and Vasculature
ER -