TY - JOUR T1 - Medical management of chronic thromboembolic pulmonary hypertension JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0107-2016 VL - 26 IS - 143 SP - 160107 AU - Joanna Pepke-Zaba AU - Hossein-Ardeschir Ghofrani AU - Marius M. Hoeper Y1 - 2017/03/31 UR - http://err.ersjournals.com/content/26/143/160107.abstract N2 - Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of acute pulmonary emboli, organised into fibrotic material that obstructs large pulmonary arteries, and distal small-vessel arteriopathy. Pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients with CTEPH; in expert centres, PEA has low in-hospital mortality rates and excellent long-term survival. Supportive medical therapy consists of lifelong anticoagulation plus diuretics and oxygen, as needed.An important recent advance in medical therapy for CTEPH is the arrival of medical therapies for patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of CTEPH in patients with inoperable disease or with recurrent/persistent pulmonary hypertension after PEA. Clinical trials of this agent have shown improvements in patients' haemodynamics and exercise capacity. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids have been used in the treatment of CTEPH, but evidence of benefit is limited. Challenges in the future development of medical therapy for CTEPH include better understanding of the underlying pathology, end-points to monitor the condition's progress, and the optimisation of pulmonary arterial hypertension therapies in relation to diverse patient characteristics and emerging options such as balloon pulmonary angioplasty.Targeted medical therapy with riociguat is beneficial for inoperable CTEPH or persistent/recurrent CTEPH after PEA http://ow.ly/pdRg3091AiF ER -