title: Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol.
abstract: Epoprostenol has markedly improved the treatment of pulmonary arterial hypertension, although predictors of outcome with epoprostenol are not well characterized. From June 1995 through August 2001, 91 patients with pulmonary arterial hypertension were treated with epoprostenol at our institution. We analyzed the effects of long-term epoprostenol treatment to determine features associated with outcome. Predictors of worse outcome included older age of disease onset (hazard ratio 3.2, 95% confidence interval 1.32-7.76 for patients above the median age of 44 years), World Health Organization functional Class IV, either at baseline or follow-up, (3.07, 1.42-6.62 compared with functional Class I, II, and III), and scleroderma spectrum of disease (2.32, 1.08-4.99). There were no baseline or follow-up hemodynamic factors predictive of outcome. Our results indicate that treatment with epoprostenol improves survival in patients with Primary Pulmonary Hypertension compared with that predicted by the National Institutes of Health Primary Pulmonary Hypertension Registry's survival equation and that their survival is significantly better than that of patients with scleroderma spectrum of disease (p = 0.001). Older patients treated with epoprostenol have significantly shorter survival, regardless of etiology.
authors.affiliation: Center for Lung Research, Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee 37232-2650, USA.
authors.forename: Karl P
authors.initials: KP
authors.lastname: Kuhn
authors.forename: Daniel W
authors.initials: DW
authors.lastname: Byrne
authors.forename: Patrick G
authors.initials: PG
authors.lastname: Arbogast
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Doyle
authors.forename: James E
authors.initials: JE
authors.lastname: Loyd
authors.forename: Ivan M
authors.initials: IM
authors.lastname: Robbins
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000959:Antihypertensive Agents / Q000494:pharmacology / Q000627:therapeutic use*; D002648:Child; D011464:Epoprostenol / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D013714:Tennessee / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol