title: [Changes long term prognosis of 17 patients with pulmonary artery hypertension].
abstract: BACKGROUND
  Pulmonary artery hypertension (PAH) is a progressive disease with high mortality. Major advances had been made in the treatment of this condition during the last decade.
  
  
  OBJECTIVE
  To characterize the clinical evolution and mortality of a cohort of Chilean patients.
  
  
  METHODS
  Seventeen patients with PAH diagnosed in the last 10 years in two Chilean hospitals were enrolled. Measurements at diagnosis included hemodynamic variables and 6-minute walk test. The patients were followed clinically for 3 years and the observed mortality was compared with that predicted by the prognostic equation proposed by the historic registry of the National Institutes of Health (NIH).
  
  
  RESULTS
  The mean age of patients was 45 years and 80% had an idiopathic PAH. The mean median pulmonary artery pressure was 57 ± 15 mmHg, the cardiac index was 2.4 ± 0.7 l/min/m² and the right atrial pressure was 12 ± 8 mmHg. The 6-minute walk distance was 348 ± 98 m. All patients received anticoagulants. Eighty two percent received ambrisentan, 12% received bosentan, 29% received iloprost and 24% sildenafil. At the end of follow-up only 3 patients had died, with an observed survival rate of 88, 82 and 82% at 1, 2 and 3 years, respectively. In contrast, the survival calculated according to the predictive formula of the NIH was 67, 56 and 45%, respectively. Among surviving patients, an improvement in exercise capacity was observed after one year (p < 0.05).
  
  
  CONCLUSIONS
  The observed survival rate was significantly better than that estimated according to historical data. Furthermore, therapy was associated with an improvement in functional capacity after one year. This prognostic improvement is consistent with data of other contemporary registries published after the NIH Registry.
authors: Hospital San Juan de Dios.
authors: Andrés
authors: A
authors: Enríquez
authors: Pablo
authors: P
authors: Castro
authors: Pablo
authors: P
authors: Sepúlveda
authors: Hugo
authors: H
authors: Verdejo
authors: Douglas
authors: D
authors: Greig
authors: Luigi
authors: L
authors: Gabrielli
authors: Marcela
authors: M
authors: Ferrada
authors: Carolina
authors: C
authors: Lapostol
mesh_terms: D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016019:Survival Analysis
chemical_list: D000959:Antihypertensive Agents