Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion

J Am Coll Cardiol. 1997 Aug;30(2):343-9. doi: 10.1016/s0735-1097(97)00187-3.


Objectives: This study sought to determine the long-term effects of continuous infusion of epoprostenol (epo) therapy on survival and pulmonary artery pressure in patients with primary pulmonary hypertension (PPH).

Background: PPH is a progressive disease for which there are few effective therapies.

Methods: Patients with PPH and New York Heart Association functional class III or IV symptoms of congestive heart failure underwent right heart catheterization and Doppler-echocardiography to measure the maximal systolic pressure gradient between the right ventricle and right atrium (delta P) and cardiac output (CO). Doppler-echocardiography and catheterization data were compared. Patients were followed up long term with Doppler-echocardiography.

Results: Of 69 patients who went on to receive epo, 18 were followed up for > 330 days (range 330 to 700). During long-term follow-up, there was a significant reduction in delta P, which decreased from 84.1 +/- 24.1 to 62.7 +/- 18.2 (mean +/- SD, p < 0.01). A Kaplan-Meier plot of survival of our study patients demonstrated improved survival compared with that of historical control subjects. The 1-, 2- and 3-year survival rates for our patients were 80% (n = 36), 76% (n = 22) and 49% (n = 6) compared with 10- (88%, n = 31), 20- (56%, n = 27) and 30-month (47%, n = 17) survival rates in historical control subjects.

Conclusions: Patients receiving continuous infusion of epo for treatment of PPH experience a decrease in pulmonary artery pressure. Long-term follow-up of this single-center patient group demonstrated improved long-term survival during epo therapy compared with that in historical control subjects and confirms predicted improved outcomes based on shorter follow-up periods.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antihypertensive Agents / administration & dosage*
  • Blood Pressure / drug effects
  • Cardiac Catheterization
  • Cardiac Output / drug effects
  • Child
  • Echocardiography, Doppler
  • Epoprostenol / administration & dosage*
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / mortality
  • Hypertension, Pulmonary / physiopathology
  • Infusions, Parenteral
  • Male
  • Middle Aged
  • Survival Rate


  • Antihypertensive Agents
  • Epoprostenol