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The Role of Medical Therapy

Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH because it is potentially curative.1 It is therefore important that all patients with suspected CTEPH undergo operability assessment at an expert center.1

However, surgery is not an option for a certain percentage of patients: 37% of newly diagnosed patients in an international prospective registry were deemed inoperable.2 Even after PEA, up to 35% of patients develop persistent or recurrent CTEPH.3

For patients with inoperable disease, or those with persistent or recurrent CTEPH after PEA surgery, medical therapy is recommended.4

CTEPH treatment algorithm

Adapted from Galiè N et al. © European Society of Cardiology/European Respiratory Society – All Rights Reserved

Riociguat is the only approved medical therapy for inoperable, persistent or recurrent CTEPH

Riociguat is approved for the treatment of patients with inoperable, persistent or recurrent CTEPH, as recommended by European guidelines.4

Riociguat is associated with long-term patient outcome

There is a large body of evidence from large, randomized controlled trials and real-world studies demonstrating the long-term benefits of riociguat in patients with inoperable, persistent or recurrent CTEPH.5-11

In the CHEST-1 study in patients with inoperable, persistent or recurrent CTEPH, riociguat significantly improved exercise capacity (+46m in 6MWD), together with a broad range of other clinical and hemodynamic endpoints including pulmonary vascular resistance, N-terminal pro-brain natriuretic peptide levels, World Health Organization functional class (WHO FC), and Borg dyspnea score.6,10

6MWD at week 16 in patients with inoperable or persistent/recurrent CTEPH

CHEST-2: Riociguat maintained improvements in 6MWD for up to 2 years in patients with inoperable, persistent or recurrent CTEPH

Probability of survival data for CTEPH

Simonneau G et al. Lancet Respir Med 2016;4:372–80 (Figure 2A) © Elsevier – All Rights Reserved

In CHEST 2, the estimated survival rate was 97% (95% CI: 93–98) at 1 year and 93% (95% CI: 89–96) at 2 years.

Riociguat is associated with high rates of overall survival at 2 years

In a long-term extension to a 12-week, multicenter, open-label, uncontrolled Phase 2 study in patients with pulmonary arterial hypertension (PAH) or inoperable CTEPH (median treatment duration 77 months), improvements in mean 6-minute walking distance were sustained for 4 years and WHO FC was stable or had improved in 92% of patients with inoperable CTEPH at month 48.7

Improvements in mean 6MWD were sustained over 4 years in patients with CTEPH7

A substantial proportion of patients with CTEPH show an inadequate clinical response to PAH therapies.11 Switching from PAH therapies to riociguat may improve exercise capacity and symptoms in patients with inoperable, persistent or recurrent CTEPH,11 and may help patients to achieve their personal treatment goals.

Evidence from the real-world CAPTURE and EXPERT studies further suggests that switching patients to riociguat from other PH-targeted therapies can be carried out safely, and is well tolerated.5,13


Riociguat is recommended in symptomatic patients who have been classified as having persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH by a CTEPH team including at least one experienced PEA surgeon4


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1.Jenkins D et al. Eur Respir J 2012;21:32–9. 2.Pepke-Zaba J et al. Circulation 2011;124:1973–81. 3.Edward JA, Mandras S. Curr Probl Cardiol 2017;42:7–38. 4.Galiè N et al. Eur Respir J 2015;46:903–75. 5.Gall H et al. Lung 2018;196:305–12. 6.Ghofrani HA et al. N Engl J Med 2013;369:319–29. 7.Halank M et al. Respir Med 2017;128:50–6. 8.Simonneau G et al. Eur Respir J 2015;45:1293–1302. 9.Simonneau G et al. Lancet Respir Med 2016;4:372–80. 10.Kim NH et al. Heart 2017;103:599–606. 11.McLaughlin VV et al. BMC Pulm Med 2017;17:216–25.12.Davey R et al. Pulm Circ 2017;7:539–42. 13.Klose H et al. Am J Respir Crit Care Med 2018;197:A5682.