PMID- 8053614 OWN - NLM STAT- MEDLINE DCOM- 19940908 LR - 20190619 IS - 0003-4819 (Print) IS - 0003-4819 (Linking) VI - 121 IP - 6 DP - 1994 Sep 15 TI - Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. PG - 409-15 AB - OBJECTIVE: To evaluate the effects of long-term-intravenous infusion of prostacyclin on exercise capacity, hemodynamics, and survival in patients with primary pulmonary hypertension. DESIGN: Open, multicenter, uncontrolled trial. SETTING: Four referral centers. PATIENTS: 18 patients with primary pulmonary hypertension: 1 New York Heart Association (NYHA) class II patient, 13 NYHA class III patients, and 4 NYHA class IV patients. INTERVENTIONS: Continuous intravenous prostacyclin administered by portable infusion pumps. All patients were treated with anticoagulant agents. MEASUREMENTS AND MAIN RESULTS: With the 6-minute walk used to evaluate exercise capacity, patients could walk on average more than 100 meters farther after prostacyclin therapy was initiated (distance at 6 months, 370 +/- 119 meters compared with 264 +/- 160 meters at baseline; P < 0.001; distance at 18 months, 408 +/- 138 meters; P = 0.02 compared with baseline). Hemodynamics were improved at 6 months: The cardiac index increased 18% (95% CI, 0.1% to 36.7%; P = 0.02), and mean pulmonary artery pressure and total pulmonary resistance decreased 9% (CI, 1.4% to 15.7%; P = 0.03) and 26% (CI, 6.1% to 46.3%; P = 0.02), respectively, compared with baseline. The improvements in cardiac index and total pulmonary resistance were maintained at 12 months (27% increase [CI, 1.3% to 51.9%; P = 0.05] and 32% decrease [CI, 9.7% to 53.6%; P = 0.02] compared with baseline, respectively). Survival was improved in NYHA class III and IV patients who received continuous prostacyclin (n = 17; follow-up, 37 to 69 months) when compared with historical controls who received standard therapy (National Institutes of Health Primary Pulmonary Hypertension Registry, n = 31, P = 0.045). Kaplan-Meier estimates of 1-, 2-, and 3-year survival rates for the patients treated with prostacyclin were 86.9%, 72.4%, and 63.3%, respectively, compared with 77.4%, 51.6%, and 40.6% for the historical control group (hazard ratio, 2.9 [CI, 1.0 to 8.0; P = 0.045]). Serious complications attributable to the drug and delivery system included two deaths and seven episodes of nonfatal sepsis in three patients. CONCLUSIONS: Continuous intravenous prostacyclin resulted in sustained clinical and hemodynamic improvement and probably in improved survival in patients with severe primary pulmonary hypertension. Despite potentially serious complications, long-term prostacyclin may be especially helpful in seriously ill patients awaiting transplantation. FAU - Barst, R J AU - Barst RJ AD - Columbia University College of Physicians and Surgeons, New York, New York. FAU - Rubin, L J AU - Rubin LJ FAU - McGoon, M D AU - McGoon MD FAU - Caldwell, E J AU - Caldwell EJ FAU - Long, W A AU - Long WA FAU - Levy, P S AU - Levy PS LA - eng PT - Clinical Trial PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - DCR9Z582X0 (Epoprostenol) SB - IM CIN - Ann Intern Med. 1994 Sep 15;121(6):463-4. PMID: 8053622 MH - Adult MH - Aged MH - Epoprostenol/*administration & dosage/adverse effects MH - Exercise MH - Female MH - Hemodynamics/drug effects MH - Humans MH - Hypertension, Pulmonary/*drug therapy/mortality/physiopathology MH - Infusions, Intravenous MH - Male MH - Middle Aged MH - Random Allocation MH - Survival Rate EDAT- 1994/09/15 00:00 MHDA- 1994/09/15 00:01 CRDT- 1994/09/15 00:00 PHST- 1994/09/15 00:00 [pubmed] PHST- 1994/09/15 00:01 [medline] PHST- 1994/09/15 00:00 [entrez] AID - 10.7326/0003-4819-121-6-199409150-00003 [doi] PST - ppublish SO - Ann Intern Med. 1994 Sep 15;121(6):409-15. doi: 10.7326/0003-4819-121-6-199409150-00003.