title: Prognostic factors in severe pulmonary hypertension patients who need parenteral prostanoid therapy: the impact of late referral.
abstract: BACKGROUND
  Oral drugs have made the treatment of pulmonary hypertension (PH) feasible in non-expert centers, which could delay patient access to prostanoid therapy.
  
  
  METHODS
  Fifty-seven consecutive patients with precapillary PH received a prostanoid in our center. Data at prostanoid initiation included modality of center referral, medical history, New York Heart Association [NYHA] class, exercise capacity, echocardiographic parameters, and hemodynamics.
  
  
  RESULTS
  Overall survival at 1, 2, and 3 years was 85%, 69%, 55%, respectively. Non-survivors had worse NYHA class III/IV (17/12) than survivors (27/1; p < 0.01) and exercise capacity on 6-minute-walk distance (254 ± 114 vs 354 ± 91 meters; p < 0.01). Non-survivors were more frequently referred on oral therapy (83% vs 36%; p < 0.01) and had a higher rate of urgent prostanoid treatment (69% vs 17%; p < 0.0001). Multivariate analysis (hazard ratio [95% confidence interval]) found the independent prognostic factors were urgent prostanoid therapy (2.0 [1.1-3.9]) and NYHA class (3.5 [1.5-8.2]). Survivors had a significant response to prostanoid, improving NYHA class from 2.8 ± 0.4 to 2.3 ± 0.5 (p = 0.002), 6-minute walk distance from 354 ± 91 to 426 ± 82 meters (p = 0.0001), and pulmonary hemodynamics (pulmonary artery pressure from 56 ± 13 to 44 ± 18 mm Hg [p < 0.05]; cardiac index from 2.0 ± 1.2 to 3.1 ± 1.2 liters/min/m(2) [p = 0.002], and pulmonary vascular resistance from 17 ± 10 to 8 ± 6 WU [p = 0.001]).
  
  
  CONCLUSIONS
  Referral of patients on oral treatment to a tertiary PH center is delayed and significantly affects prognosis.
authors.affiliation: Department of Cardiovascular and Respiratory Science, I School of Medicine, University of Rome Sapienza, Policlinico Umberto I.
authors.forename: Roberto
authors.initials: R
authors.lastname: Badagliacca
authors.forename: Beatrice
authors.initials: B
authors.lastname: Pezzuto
authors.forename: Roberto
authors.initials: R
authors.lastname: Poscia
authors.forename: Massimo
authors.initials: M
authors.lastname: Mancone
authors.forename: Silvia
authors.initials: S
authors.lastname: Papa
authors.forename: Serena
authors.initials: S
authors.lastname: Marcon
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valli
authors.forename: Gennaro
authors.initials: G
authors.lastname: Sardella
authors.forename: Fabio
authors.initials: F
authors.lastname: Ferrante
authors.forename: Carlo
authors.initials: C
authors.lastname: Iacoboni
authors.forename: Daniela
authors.initials: D
authors.lastname: Parola
authors.forename: Francesco
authors.initials: F
authors.lastname: Fedele
authors.forename: Carmine Dario
authors.initials: CD
authors.lastname: Vizza
mesh_terms: D000328:Adult; D000368:Aged; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics / Q000187:drug effects / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality* / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011453:Prostaglandins / Q000494:pharmacology / Q000627:therapeutic use*; D012017:Referral and Consultation; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D011453:Prostaglandins; D011464:Epoprostenol; C427248:treprostinil