PMID- 10445611
OWN - NLM
STAT- MEDLINE
DCOM- 19990917
LR  - 20220318
IS  - 0903-1936 (Print)
IS  - 0903-1936 (Linking)
VI  - 13
IP  - 6
DP  - 1999 Jun
TI  - Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary 
      hypertension secondary to connective tissue diseases: results of a pilot study.
PG  - 1351-6
AB  - Continuous intravenous epoprostenol improves exercise capacity, haemodynamics, 
      and survival in severe primary pulmonary hypertension. Pulmonary hypertension can 
      also be life-threatening in patients with connective tissue diseases. In a 
      prospective open monocentre uncontrolled study, the effects of epoprostenol were 
      evaluated in patients with severe pulmonary hypertension secondary to connective 
      tissue diseases who were unresponsive to oral vasodilators (including calcium 
      channel blockers) and continued to be in the New York Heart Association (NYHA) 
      functional class III or IV despite conventional medical therapy. Seventeen 
      patients received epoprostenol administered by a portable infusion pump 
      associated with conventional therapy (oral anticoagulants, diuretics, 
      supplemental oxygen). During the first six weeks of therapy, two (12%) patients 
      died, of pulmonary oedema (n = 1) and severe sepsis (n = 1). In the fifteen 
      remaining subjects, clinical and haemodynamic parameters improved significantly 
      at six weeks. These patients were subsequently monitored for 80+/-48 (range 
      14-154) weeks after initiation of epoprostenol. Five (33%) patients died, of 
      right heart failure (n = 2), severe sepsis (n = 2) or syncope (n = 1) and two 
      patients were successfully transplanted 24 and 52 weeks after initiation of 
      epoprostenol. Seven of the remaining eight patients had a persistent clinical 
      improvement. Short-term epoprostenol therapy is effective in some patients with 
      connective tissue diseases as demonstrated by better clinical status and 
      haemodynamics at six weeks. However, this study reports several cases of early 
      and late major complications including severe sepsis and pulmonary oedema. 
      Additional information is needed to evaluate the benefit: risk ratio of long-term 
      epoprostenol therapy in pulmonary hypertension secondary to connective tissue 
      diseases.
FAU - Humbert, M
AU  - Humbert M
AD  - Service de Pneumologie et Reanimation Respiratoire, UPRES EA2705, Hopital Antoine 
      Beclere, Clamart, France.
FAU - Sanchez, O
AU  - Sanchez O
FAU - Fartoukh, M
AU  - Fartoukh M
FAU - Jagot, J L
AU  - Jagot JL
FAU - Le Gall, C
AU  - Le Gall C
FAU - Sitbon, O
AU  - Sitbon O
FAU - Parent, F
AU  - Parent F
FAU - Simonneau, G
AU  - Simonneau G
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - Eur Respir J
JT  - The European respiratory journal
JID - 8803460
RN  - 0 (Antihypertensive Agents)
RN  - DCR9Z582X0 (Epoprostenol)
SB  - IM
MH  - Adult
MH  - Antihypertensive Agents/*administration & dosage
MH  - Connective Tissue Diseases/*complications
MH  - Drug Administration Schedule
MH  - Epoprostenol/*administration & dosage
MH  - Exercise Tolerance
MH  - Female
MH  - Hemodynamics
MH  - Humans
MH  - Hypertension, Pulmonary/drug therapy/etiology/mortality/physiopathology
MH  - Infusions, Intravenous
MH  - Male
MH  - Middle Aged
MH  - Pilot Projects
MH  - Prospective Studies
MH  - Survival Rate
EDAT- 1999/08/13 00:00
MHDA- 1999/08/13 00:01
CRDT- 1999/08/13 00:00
PHST- 1999/08/13 00:00 [pubmed]
PHST- 1999/08/13 00:01 [medline]
PHST- 1999/08/13 00:00 [entrez]
AID - 10.1183/09031936.99.13613579 [doi]
PST - ppublish
SO  - Eur Respir J. 1999 Jun;13(6):1351-6. doi: 10.1183/09031936.99.13613579.