title: Survival in primary pulmonary hypertension: the impact of epoprostenol therapy.
abstract: BACKGROUND
  Primary pulmonary hypertension (PPH) is a severe and progressive disease. Without treatment, the median survival is 2.8 years, with survival rates of 68%, 48%, and 34% at 1, 3, and 5 years, respectively. Intravenous epoprostenol was the first Food and Drug Administration-approved therapy for PPH. The long-term impact that epoprostenol has made on PPH remains to be defined.
  
  
  RESULTS
  One hundred sixty-two consecutive patients diagnosed with PPH and treated with epoprostenol were followed for a mean of 36.3 months (median, 31 months). Data including functional class, exercise tolerance, and hemodynamics were recorded in a customized database. Vital status was verified in each patient. Observed survival with epoprostenol therapy at 1, 2, and 3 years was 87.8%, 76.3%, and 62.8% and was significantly greater than the expected survival of 58.9%, 46.3%, and 35.4% based on historical data. Baseline predictors of survival included exercise tolerance, functional class, right atrial pressure, and vasodilator response to adenosine. Predictors of survival after the first year of therapy included functional class and improvement in exercise tolerance, cardiac index, and mean pulmonary artery pressure.
  
  
  CONCLUSIONS
  Intravenous epoprostenol improves long-term survival in PPH.
authors.affiliation: Rush-Presbyterian-St Luke's Medical Center, Department of Medicine, Section of Cardiology, Chicago, Ill 60612, USA. vmclaugh@rush.edu
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
authors.forename: Alicia
authors.initials: A
authors.lastname: Shillington
authors.forename: Stuart
authors.initials: S
authors.lastname: Rich
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011464:Epoprostenol / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality*; D008297:Male; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol