title: Medical management of chronic thromboembolic pulmonary hypertension.
abstract: 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.
authors.affiliation: Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, UK Joanna.PepkeZaba@papworth.nhs.uk.
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.affiliation: Universities of Giessen and Marburg Lung Center, Giessen, Germany.
authors.forename: Hossein-Ardeschir
authors.initials: HA
authors.lastname: Ghofrani
authors.affiliation: Dept of Respiratory Medicine, Hannover Medical School and Center for Lung Research, Hannover, Germany.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
mesh_terms: D000925:Anticoagulants / Q000009:adverse effects / Q000627:therapeutic use*; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D002908:Chronic Disease; D004232:Diuretics / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D010102:Oxygen Inhalation Therapy / Q000009:adverse effects; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D012008:Recurrence; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D004232:Diuretics