title: Chronic thromboembolic pulmonary hypertension: role of medical therapy.
abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease with poor prognosis if not treated. The treatment of choice is surgery with pulmonary endarterectomy. However, a significant percentage of patients are deemed non-operable due to distal distribution of the disease and arteriopathy in the non-occluded areas that is indistinguishable from pulmonary arterial hypertension (PAH). The overlap in clinical presentation, pathological features and pathogenesis between PAH and CTEPH provides a compelling rationale for exploring the efficacy of PAH-targeted therapies in CTEPH. These therapies are often considered for non-operable patients and are also used in operable patients as a bridge to surgery or as post-pulmonary endarterectomy therapy for persistent pulmonary hypertension, despite the fact they are not licensed for CTEPH. Two randomised clinical trials have been performed in non-operable CTEPH patients. The BENEFiT study, with the endothelin receptor antagonist bosentan, did not show improvement in walking distance. Recently, the CHEST-1 trial, with the soluble guanylate cyclase stimulator riociguat, met study end-point and demonstrated significant improvement in walking distance in patients with non-operable CTEPH. There is an urgent need for more randomised clinical trials designed to clarify whether administration of PAH-targeted therapies improves clinically meaningful end-points in various CTEPH populations.
authors: Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, UK. joanna.pepkezaba@papworth.nhs.uk
authors: Joanna
authors: J
authors: Pepke-Zaba
authors: Pavel
authors: P
authors: Jansa
authors: Nick H
authors: NH
authors: Kim
authors: Robert
authors: R
authors: Naeije
authors: Gerald
authors: G
authors: Simonneau
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D002908:Chronic Disease; D003937:Diagnosis, Differential; D018450:Disease Progression; D004691:Endarterectomy; D065128:Endothelin Receptor Antagonists; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000628:therapy*; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D011453:Prostaglandins / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D013923:Thromboembolism / Q000175:diagnosis / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins