In the past 15-20 years enormous progress has been made in the treatment of pulmonary arterial hypertension, PAH, one of the five subtypes of pulmonary hypertension (WHO group 1), with nine drugs now approved by EU regulatory authorities. The new treatments have led to significant improvements for patients in terms of symptoms, disease progression and survival.
Riociguat, a soluble guanylate cyclase stimulator, is the first approved treatment specifically for patients with chronic thromboembolic pulmonary hypertension, CTEPH (WHO group 4). It can be used for patients who have inoperable CTEPH and those with persistent or recurrent CTEPH following pulmonary endarterectomy surgery (PEA).
Follow the links on the left for details of treatment options for PAH and CTEPH.
Currently there are no approved drugs for the other three classes of pulmonary hypertension (WHO groups 2, 3 and 5). Research on new treatments in currently ongoing and may in the near future lead to new drug approvals.