Riociguat (Adempas®) is recommended as an option for restricted use within NHS Wales for the treatment of adult patients with pulmonary arterial hypertension (PAH) with WHO functional class II to III to improve exercise capacity. This recommendation applies only in circumstances where the approved Wales Patient Access Scheme is utilised. Riociguat (Adempas®) should be restricted for use as a PAH-specific monotherapy as an alternative treatment option to endothelin receptor antagonist (ERA) monotherapy in adult patients with PAH of WHO functional class II to III. Riociguat (Adempas®) is not recommended for use within NHS Wales outside of this subpopulation.