PRIMARY PULMONARY-HYPERTENSION - IMPROVED LONG-TERM EFFECTS AND SURVIVAL WITH CONTINUOUS INTRAVENOUS EPOPROSTENOL INFUSION

Objectives. This study sought to determine the long-term effects of co ntinuous infusion of epoprostenol (epo) therapy on survival and pulmon ary artery pressure in patients with primary pulmonary hypertension (P PH). Background. PPH is a progressive disease for which there are few effective therapies. Methods. Patients with PPH and New York Heart Ass ociation functional class III or IV symptoms of congestive heart failu re underwent right heart catheterization and Doppler echocardiography to measure the maximal systolic pressure gradient between the right ve ntricle and right atrium (Delta P) and cardiac output (GO). Doppler-ec hocardiography and catheterization data were compared. Patients were f ollowed up long term with Doppler-echocardiography. Results. Of 69 pat ients 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 s tudy patients demonstrated improved survival compared with that of his torical 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 wit h 10-(88%, n = 31), 20-(56%, n = 27) and 30 month (47%, n = 17) surviv al rates in historical control subjects. Conclusions. Patients receivi ng continuous infusion of epo for treatment of PPH experience a decrea se in pulmonary artery pressure. Long-term follow-up of this single-ce nter patient group demonstrated improved long-term survival during epo therapy compared with that in historical control subjects and confirm s predicted improved outcomes based on shorter follow-up periods. (C) 1997 by the American College of Cardiology.