According to the 2022 ESC/ERS guidelines for pulmonary hypertension, for patients with IPAH/HPAH/DPAH who present with an intermediate-low risk of death while receiving ERA/PDE-5i therapy, switching from PDE-5is to riociguat may be considered (IIb) (Humbert et al., 2022). The gene discussed is ALDH7A1; the disease is pulmonary arterial hypertension.