title: The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study.
abstract: The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, many patients develop a severe progressive small vessel pulmonary arteriopathy that is inaccessible to surgical intervention and is associated with poor survival. The purpose of the present study was to evaluate the medium-term efficacy and safety of the dual endothelin receptor antagonist, bosentan, in inoperable CTEPH. Forty-seven patients with inoperable CTEPH (distal disease or persistent pulmonary hypertension following PEA) underwent evaluation after 1 yr of bosentan therapy. Outcomes included assessment of 6-min walk test (6MWT), haemodynamics and World Health Organization functional classification. Monitoring of serious adverse effects and changes in therapy was undertaken. Patients showed sustained improvements in 6MWT (49+/-8 m), functional classification, cardiac index (+0.2+/-0.07 L.min(-1).m(-2)) and total pulmonary resistance (-139+/-42 dyn.s.cm(-5)). Those patients with persisting pulmonary hypertension following PEA showed the greatest improvement. One-yr survival was 96%, and bosentan was well tolerated with only one patient developing deranged liver function. Although all patients with chronic thromboembolic pulmonary hypertension should be considered for pulmonary endarterectomy, bosentan provides an alternative medical therapy to improve function and delay the progression of this devastating disease in those in whom surgery is not suitable.
authors.affiliation: Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridgeshire, CB3 8RE, UK. rodneyhughes@onetel.com
authors.forename: R J
authors.initials: RJ
authors.lastname: Hughes
authors.forename: X
authors.initials: X
authors.lastname: Jais
authors.forename: D
authors.initials: D
authors.lastname: Bonderman
authors.forename: J
authors.initials: J
authors.lastname: Suntharalingam
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.forename: I
authors.initials: I
authors.lastname: Lang
authors.forename: G
authors.initials: G
authors.lastname: Simonneau
authors.forename: J
authors.initials: J
authors.lastname: Pepke-Zaba
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000494:pharmacology; D000959:Antihypertensive Agents / Q000494:pharmacology*; D000077300:Bosentan; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013449:Sulfonamides / Q000494:pharmacology*; D013923:Thromboembolism / Q000188:drug therapy* / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan