60 50 40 30 20 10 0
Patent-2 open label study
8 Figure 2: Effect of riociguat therapy on 6MWD in PAH patients Safety and tolerability
With respect to safety and tolerability, in CHEST-2, three cases of haemoptysis were reported at years 1 and 2 (one mild, one moderate and one severe). No cases were considered to be study drug-related.17 Data from the PATENT-1 and PATENT-2 study did report haemoptysis in five patients; however, no cases of haemoptysis were considered to be related to riociguat treatment. One out of two occurrences of pulmonary haemorrhage was considered related to the study drug.18 In addition to the two study drug-related severe adverse events that were fatal in the first year of PATENT-2 (one of PAH and one of pulmonary haemorrhage), there was one new case of haemoptysis in the second year of PATENT-2 that was fatal and considered study drug-related.19
Conclusions Riociguat is a first-in-class medication, and was approved for the treatment of inoperable CTEPH or persistent PH following pulmonary endarterectomy in 2013. It is the first drug to be approved for this indication. It is important to recognise that pulmonary endarterectomy remains the treatment of choice for CTEPH patients; in patients eligible for surgical intervention, riociguat is not currently indicated. Medical therapy with riociguat should not delay or replace surgical intervention when feasible. In addition, there are no other PAH-specific therapies currently approved for the use in patients with persistent PH following endarterectomy or inoperable CTEPH. Ongoing clinical trials are evaluating the use of riociguat in other types of PH,
including PH associated with lung disease and PH associated with left heart dysfunction (preserved and reduced ejection fraction) has been investigated. Until the results of these clinical trials, riociguat should not be prescribed in these groups of patients. l
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Change from baseline in six-minute walk distance (m)
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