PMID- 20209595
OWN - NLM
STAT- MEDLINE
DCOM- 20100706
LR  - 20131121
IS  - 1527-6473 (Electronic)
IS  - 1527-6465 (Linking)
VI  - 16
IP  - 3
DP  - 2010 Mar
TI  - Acute and long-term effects of inhaled iloprost in portopulmonary hypertension.
PG  - 348-56
LID - 10.1002/lt.21997 [doi]
AB  - Portopulmonary hypertension (PoPH) is a serious condition without an established 
      treatment. Drugs used to treat pulmonary hypertension may have detrimental 
      effects on portal hypertension. This study was designed to assess in patients 
      with PoPH the acute effects of inhaled iloprost (iILO) on pulmonary and hepatic 
      hemodynamics and to evaluate the clinical outcome after 12 months of treatment. 
      We conducted 2 separate studies. In the first one, 21 patients with PoPH were 
      acutely tested with 2.8 microg of iILO. Pulmonary and hepatic hemodynamics were 
      assessed at the baseline and through 60 minutes after iILO. In the second one, we 
      retrospectively evaluated 12 patients treated with iILO (30 microg/day) for more 
      than 1 year. The 6-minute walk distance (6MWD), functional class (FC), and 
      echocardiogram were analyzed at the baseline and after 12 months of treatment. In 
      the acute study, iILO rapidly reduced pulmonary artery pressure (PAP; -16% + or - 
      8%, P < 0.001) and pulmonary vascular resistance (-18% + or - 14%, P < 0.001). 
      The cardiac output did not change initially but decreased after 30 minutes. The 
      hepatic venous pressure gradient (HVPG) and hepatic blood flow did not vary 
      through the study. Pulmonary vasodilation induced by iILO was inversely related 
      to HVPG. In the long-term evaluation, iILO improved FC by 1 or more in 7 patients 
      (P = 0.04) and increased 6MWD by 67 + or - 59 m at 12 months (P < 0.001). No 
      change in systolic PAP was observed. Two patients died because of hepatic 
      complications, and 4 additional patients presented clinically significant events 
      that were related to hepatic disease in 2 and worsening of pulmonary hypertension 
      in 2. We conclude that in patients with PoPH, iILO produces rapid and selective 
      pulmonary vasodilation without altering the hepatic hemodynamics. Its long-term 
      use may provide sustained improvements in symptoms and exercise tolerance in some 
      patients with PoPH. A randomized, controlled trial is warranted to establish its 
      clinical role in this serious condition.
FAU - Melgosa, Maria Teresa
AU  - Melgosa MT
AD  - Department of Pulmonary Medicine, University of Barcelona, Barcelona, Spain.
FAU - Ricci, Giovanni L
AU  - Ricci GL
FAU - Garcia-Pagan, Juan Carlos
AU  - Garcia-Pagan JC
FAU - Blanco, Isabel
AU  - Blanco I
FAU - Escribano, Pilar
AU  - Escribano P
FAU - Abraldes, Juan G
AU  - Abraldes JG
FAU - Roca, Josep
AU  - Roca J
FAU - Bosch, Jaume
AU  - Bosch J
FAU - Barbera, Joan Albert
AU  - Barbera JA
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Liver Transpl
JT  - Liver transplantation : official publication of the American Association for the 
      Study of Liver Diseases and the International Liver Transplantation Society
JID - 100909185
RN  - 0 (Vasodilator Agents)
RN  - JED5K35YGL (Iloprost)
SB  - IM
MH  - Administration, Inhalation
MH  - Adult
MH  - Blood Pressure/drug effects/physiology
MH  - Cohort Studies
MH  - Dose-Response Relationship, Drug
MH  - Female
MH  - Humans
MH  - Hypertension, Portal/*drug therapy/physiopathology
MH  - Hypertension, Pulmonary/*drug therapy/physiopathology
MH  - Iloprost/administration & dosage/pharmacology/*therapeutic use
MH  - Liver Circulation/drug effects/physiology
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Vascular Resistance/drug effects/physiology
MH  - Vasodilator Agents/administration & dosage/pharmacology/*therapeutic use
EDAT- 2010/03/09 06:00
MHDA- 2010/07/07 06:00
CRDT- 2010/03/09 06:00
PHST- 2010/03/09 06:00 [entrez]
PHST- 2010/03/09 06:00 [pubmed]
PHST- 2010/07/07 06:00 [medline]
AID - 10.1002/lt.21997 [doi]
PST - ppublish
SO  - Liver Transpl. 2010 Mar;16(3):348-56. doi: 10.1002/lt.21997.