Treprostinil for the treatment of chronic thromboembolic pulmonary hypertension

Introduction: Parenteral treprostinil for patients with pulmonary arterial hypertension has resulted in improvement of exercise capacity, functional class, hemodynamics, and survival. Recently, a first randomized trial performed in patients with chronic thromboembolic pulmonary hypertension confirmed the efficacy of subcutaneous treprostinil in this subset of pulmonary hypertension.Areas covered: Treprostinil sodium is a prostacyclin analog produced synthetically. Drug characteristics include potent systemic and pulmonary vasodilatory effects. Local side-effects of subcutaneous treprostinil have been an obstacle for its use. However, in contrast to other prostacyclins, treprostinil has favorable features. We performed a literature survey by searching PubMed for clinical trials published in any language, investigating medicinal treatments for CTEPH. We used the search terms ‘inoperable’, and ‘chronic thromboembolic pulmonary hypertension’ with ‘randomized clinical trial’, and have put treprostinil for CTEPH in the contest of published literature.Expert opinion: Drugs approved for PAH have recently shown excellent efficacy in patients with non-operable CTEPH. Rather than head-to-head comparisons of drugs, combination treatments are to be expected in the near future. Furthermore, drugs will have to be tested alongside with pulmonary endarterectomy (PEA), and alongside balloon pulmonary angioplasty, a promising percutaneous mechanical treatment for CTEPH that is not suited for PEA.