Recently, riociguat, a stimulator of soluble guanylate synthase (sGC) has been approved for treatment of pulmonary arterial hypertension and chronic thromboembolic PH after it showed improved 6‐minute walk distance, compared with placebo, and also improved PVR, functional class, dyspnea, and health‐related quality of life in these diseases.29, 30 riociguat was also investigated in a single dose study with patients with COPD with borderline or manifest PH (mPAP ≥23 mmHg).31 Similar as for other therapies, significant reductions of mPAP and PVR could be demonstrated in this patient population. This evidence concerns the gene SGCB and pulmonary arterial hypertension.