title: New predictors of outcome in idiopathic pulmonary arterial hypertension.
abstract: Idiopathic pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis. New therapies have improved the outcome of this condition; accordingly, the factors that determine outcome may have changed. We aimed to identify determinants of survival in a cohort of consecutive patients with PAH: which was idiopathic, familial, or associated with anorexigen use. We performed a retrospective cohort study of 84 consecutive patients with PAH who underwent initial evaluation at our center from January 1994 to June 2002. The primary outcome was death or lung transplantation. Survival at 1, 3, and 5 [corrected] years was 87%, 75%, and 61%, respectively. Multivariate analysis showed that being of African-American or Asian descent was associated with an increased risk of death. Warfarin use was associated with a reduced risk of death. Higher serum albumin and cardiac index and acute vasoreactivity were independently associated with improved survival. These data suggest that the determinants of outcome have changed. Race is identified as a new risk factor, which may be attributable to biologic or socioeconomic differences. Cardiac function and acute reactivity of the pulmonary vascular bed remain strong independent predictors of outcome.
authors.affiliation: Department of Internal Medicine, Joseph L. Mailman School of Public Health, Columbia University, 622 West 168th Street, New York, NY 10032, USA. sk2097@columbia.edu
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.forename: Evelyn M
authors.initials: EM
authors.lastname: Horn
authors.forename: Ketevan K
authors.initials: KK
authors.lastname: Berekashvili
authors.forename: Robert P
authors.initials: RP
authors.lastname: Garofano
authors.forename: Rochelle L
authors.initials: RL
authors.lastname: Goldsmith
authors.forename: Allison C
authors.initials: AC
authors.lastname: Widlitz
authors.forename: Erika B
authors.initials: EB
authors.lastname: Rosenzweig
authors.forename: Diane
authors.initials: D
authors.lastname: Kerstein
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
mesh_terms: D000328:Adult; D044466:Asian People; D044383:Black People; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology / Q000235:genetics / Q000401:mortality / Q000473:pathology; D008297:Male; D009519:New York City / Q000453:epidemiology; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000473:pathology*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D016019:Survival Analysis; D001741:Black or African American