Treating pulmonary hypertension in COPD: where do we start?

E ndothelin receptor antagonists, phosphodiesterase (PDE)-5 inhibitors and prostanoids are effective treatments for pulmonary arterial hypertension (PAH), a severe but relatively rare form of pulmonary hypertension [1–3]. Other forms of pulmonary hypertension as seen, for instance, in chronic lung disease are much more common than PAH and although usually less severe, they are clinically meaningful as they are associated with aggravated symptoms and shorter survival [4, 5]. Thus, it comes as no surprise that physicians are tempted to use so-called PAH drugs in other forms of pulmonary hypertension, even though there is limited evidence supporting this approach.