title: Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation.
abstract: The present report describes two patients with long-term survival after being diagnosed with idiopathic pulmonary arterial hypertension more than 20 years earlier. Both patients were treated with calcium channel blockers for several years and are currently maintained on bosentan, an oral endothelin receptor antagonist. Severe dilation of the main pulmonary arteries is present in both patients and may be related to long-term survival with idiopathic pulmonary arterial hypertension.
authors.affiliation: Department of Medicine, University of British Columbia, Vancouver.
authors.forename: Vanja
authors.initials: V
authors.lastname: Petrovic
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Ryerson
authors.forename: Robert D
authors.initials: RD
authors.lastname: Levy
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization / Q000379:methods; D004108:Dilatation, Pathologic; D004452:Echocardiography / Q000379:methods; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000473:pathology / Q000503:physiopathology; D011652:Pulmonary Circulation / Q000187:drug effects*; D011859:Radiography; D013449:Sulfonamides / Q000627:therapeutic use*; D017741:Survivors; D013927:Thrombosis / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan