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Chronic thromboembolic pulmonary hypertension is essentially a surgical disease, curable by pulmonary endarterectomy, with acceptable procedural mortality in experienced centres. Patient selection for surgery is extremely complex and results in 30\u201350% of patients considered inoperable. A large clinical experience has been built up with endothelin receptor antagonists and phosphodiesterase-5 inhibitors, while evidence from controlled trials is running far behind schedule. More recently, a randomised controlled trial with the guanylate cyclase stimulator, riociguat, achieved its target and showed haemodynamic, as well as functional, improvements within 4 months of therapy. 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