title: Five-Year outcomes of patients enrolled in the REVEAL Registry.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a rare, severe disease characterized by worsening right-sided heart failure, decreasing functional status, and poor survival. The present study characterizes the 5-year survival in the United States of a new and previous diagnosis of PAH in patients stratified by baseline functional class (FC). The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) is a 55-center observational US registry of the demographics, disease course, and management of patients with World Health Organization (WHO) group 1 PAH.
  
  
  METHODS
  The REVEAL Registry enrolled newly and previously diagnosed patients aged ≥ 3 months with WHO group 1 PAH consecutively from March 2006 to December 2009. Demographics, disease characteristics, and hemodynamic data were collected at enrollment. Survival analysis was conducted by FC and other subgroups in patients aged ≥ 18 years.
  
  
  RESULTS
  Survival differences between previously diagnosed and newly diagnosed patients at 1 year (90.4% vs 86.3%) were maintained to 5 years; 5-year survival for previously diagnosed patients was 65.4% compared with 61.2% for newly diagnosed patients. Previously diagnosed patients in FC I, II, III, and IV had an estimated 5-year survival rate of 88.0%, 75.6%, 57.0%, and 27.2%, respectively, compared with 72.2%, 71.7%, 60.0%, and 43.8% for newly diagnosed patients in FC I, II, III, and IV, respectively.
  
  
  CONCLUSIONS
  Patient survival of advanced PAH remains poor at 5 years despite treatment advances. New York Heart Association FC remains one of the most important predictors of future survival. These observations reinforce the importance of continuous monitoring of FC in patients with PAH.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
authors.forename: Harrison W
authors.initials: HW
authors.lastname: Farber
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.forename: Abby D
authors.initials: AD
authors.lastname: Poms
authors.forename: David B
authors.initials: DB
authors.lastname: Badesch
authors.forename: Adaani E
authors.initials: AE
authors.lastname: Frost
authors.forename: Erwan
authors.initials: E
authors.lastname: Muros-Le Rouzic
authors.forename: Alain J
authors.initials: AJ
authors.lastname: Romero
authors.forename: Wade W
authors.initials: WW
authors.lastname: Benton
authors.forename: C Gregory
authors.initials: CG
authors.lastname: Elliott
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
authors.forename: Raymond L
authors.initials: RL
authors.lastname: Benza
mesh_terms: D000368:Aged; D019468:Disease Management; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014481:United States / Q000453:epidemiology