title: Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry.
abstract: OBJECTIVE
  New York Heart Association/World Health Organization functional class (FC) is associated with outcomes in pulmonary arterial hypertension (PAH). We assessed whether patients with PAH who improve from FC III to FC I/II have improved survival vs patients who remain at FC III or worsen to FC IV.
  
  
  METHODS
  Patients aged ≥ 19 years with FC III PAH from the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry) (N = 982) were categorized as improved, unchanged, or worsened according to their change in FC from enrollment to first follow-up assessment within 1 year of enrollment. Kaplan-Meier estimates of 3-year survival from first follow-up and changes in 6-min walk distance (6MWD) from enrollment to first follow-up were determined. Subgroup analyses were conducted by cause (ie, idiopathic/familial, connective tissue disease [CTD], congenital heart disease) and time of diagnosis (ie, newly or previously diagnosed [diagnostic right-sided heart catheterization within or ≥3 months of enrollment, respectively]).
  
  
  RESULTS
  Overall, 27% of patients improved FC. Survival was better in patients whose FC improved (84%±2%, n=263) vs those who remained unchanged (66%±2%, n=645) or worsened (29%±6%, n=74) (all P<.001). Survival was also better in patient subgroups whose FC improved vs those who remained unchanged (idiopathic/familial [P<.001], CTD-associated PAH [P=.009], whether newly [P=.004] or previously diagnosed [P<.001]). 6MWD improvements were greater in patients whose FC improved vs those who remained unchanged in the overall (P<.001) and CTD (P=.028) cohorts.
  
  
  CONCLUSIONS
  Patients with PAH who improve from FC III to I/II, whether newly or previously diagnosed and regardless of PAH cause, have better survival vs patients who remain FC III.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
authors: Columbia University College of Physicians and Surgeons, New York, NY.
authors: Robyn J
authors: RJ
authors: Barst
authors: Stanford University School of Medicine, Stanford, CA; Palo Alto VA Health Care System, Palo Alto, CA.
authors: Lorinda
authors: L
authors: Chung
authors: Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford, CA.
authors: Roham T
authors: RT
authors: Zamanian
authors: ICON Late Phase & Outcomes Research, San Francisco, CA.
authors: Michelle
authors: M
authors: Turner
authors: Mayo Clinic, Rochester, MN. Electronic address: mmcgoon@mayo.edu.
authors: Michael D
authors: MD
authors: McGoon
mesh_terms: D000328:Adult; D000368:Aged; D019468:Disease Management; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification* / Q000401:mortality* / Q000628:therapy; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome