| Summary: | Riociguat, a soluble guanylate cyclase stimulator (sGC), is currently the only drug with Health Canada approved indication for the management of inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent or recurrent CTEPH after surgical treatment in adults 18 years or older with progressive pulmonary hypertension (PH) of World Health Organization (WHO) functional class II or III. The WHO functional class ranges from I to IV, with higher numbers indicating greater functional limitations. Since histopathology studies suggest that CTEPH and primary pulmonary artery hypertension (PAH) share common pathways in their pathophysiology and manifest similar small-vessel changes, vasoactive therapies with evidence of efficacy in PAH are commonly prescribed off-label for CTEPH patients who require medical intervention. In Canada, specific therapies for PAH include sGC stimulators, endothelin receptor antagonists (ERA), phosphodiesterase-5 inhibitors (PDE-5), and prostacyclin analogues. The objective of this report is to review current evidence of comparative efficacy and safety of monotherapy or combination therapy of medical interventions for patients with CTEPH.
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