title: Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension.
abstract: Pathophysiological findings in chronic thromboembolic pulmonary hypertension (CTEPH) have suggested that a secondary small vessel arteriopathy may contribute to the haemodynamic impairment observed in these patients. It was hypothesised that this element of the elevated vascular resistance may be responsive to continuous intravenous epoprostenol therapy. Retrospectively, the clinical and haemodynamic responses to continuous intravenous epoprostenol were evaluated in nine CTEPH patients who subsequently underwent pulmonary thromboendarterectomy (PTE). Cardiopulmonary haemodynamics were determined prior to the initiation of epoprostenol, while on epoprostenol, prior to PTE, and after PTE. Six patients, treated for 2-26 months prior to PTE, experienced either clinical stability or improvement that was associated with a mean reduction in pulmonary vascular resistance (PVR) of 28% (median 33%, range 0-46%). Three patients, treated for 3-9 months, experienced clinical deterioration during epoprostenol administration, with a significant increase in PVR in two patients. Subsequent PTE resulted in a highly significant improvement of cardiac index, mean pulmonary artery pressure and total pulmonary resistance. To conclude, selected patients with chronic thromboembolic pulmonary hypertension may benefit clinically and haemodynamically from continuous intravenous epoprostenol treatment prior to pulmonary thromboendarterectomy. Factors predictive of a beneficial response, and whether this intervention influences either morbidity or mortality associated with pulmonary thromboendarterectomy, remain to be established.
authors.affiliation: Academic Medical Centre, University of Amsterdam, Dept of Pulmonology, F5-162, PO Box 22700, 1100 DE Amsterdam, The Netherlands. p.bresser@amc.uva.nl
authors.forename: P
authors.initials: P
authors.lastname: Bresser
authors.forename: P F
authors.initials: PF
authors.lastname: Fedullo
authors.forename: W R
authors.initials: WR
authors.lastname: Auger
authors.forename: R N
authors.initials: RN
authors.lastname: Channick
authors.forename: I M
authors.initials: IM
authors.lastname: Robbins
authors.forename: K M
authors.initials: KM
authors.lastname: Kerr
authors.forename: S W
authors.initials: SW
authors.lastname: Jamieson
authors.forename: L J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000328:Adult; D001794:Blood Pressure / Q000187:drug effects; D002302:Cardiac Output / Q000187:drug effects; D002908:Chronic Disease; D004691:Endarterectomy; D011464:Epoprostenol / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D011651:Pulmonary Artery / Q000503:physiopathology; D011655:Pulmonary Embolism / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D010975:Platelet Aggregation Inhibitors; D011464:Epoprostenol