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

M C J van Thor; L Ten Klooster; R J Snijder; M C Post; J J Mager

doi:10.1016/j.ijcha.2019.02.004

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

Int J Cardiol Heart Vasc. 2019 Feb 28:22:163-168. doi: 10.1016/j.ijcha.2019.02.004. eCollection 2019 Mar.

Authors

M C J van Thor^{1

2}, L Ten Klooster², R J Snijder², M C Post¹, J J Mager²

Affiliations

¹ Dept of Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands.
² Dept of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.

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.

Keywords: 6MWD, 6-minute walking distance; AE, adverse event; BPA, balloon pulmonary angioplasty; CHEST, Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate Cyclase-Stimulator Trial; CO, cardiac output; CTEPH, chronic thromboembolic pulmonary hypertension; CW, clinical worsening; Chronic thromboembolic pulmonary hypertension; Clinical outcome; Clinical worsening; ERA, endothelin receptor antagonist; FC, functional class; HR, hazards regression; 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; Riociguat; SD, standard deviation; Survival; WHO, World Health Organization; e.g., exempli gratiā; i.e., id est; mPAP, mean pulmonary arterial pressure; sGC, soluble guanylate cyclase.