Elsevier

IJC Heart & Vasculature

Volume 22, March 2019, Pages 163-168
IJC Heart & Vasculature

Long-term clinical value and outcome of riociguat in chronic thromboembolic pulmonary hypertension

https://doi.org/10.1016/j.ijcha.2019.02.004Get rights and content
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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.

Abbreviations

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

Keywords

Chronic thromboembolic pulmonary hypertension
Riociguat
Clinical outcome
Survival
Clinical worsening

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