Plasma cytokines and portopulmonary hypertension in patients with cirrhosis waiting for orthotopic liver transplantation

Angiology. 2010 Nov;61(8):802-6. doi: 10.1177/0003319710369101. Epub 2010 May 24.


Portopulmonary hypertension (PPHTN) is a rare complication in patients with portal hypertension. A role of endothelin 1 (ET-1) and other cytokines was demonstrated in primary pulmonary hypertension but not in PPHTN. We evaluated the possible role of ET-1, interleukin 6 (IL-6), interleukin 1β (IL-1β), and tumor necrosis factor alpha (TNF-α) in the pathogenesis of PPHTN. Plasmatic concentrations of ET-1, IL-6, IL-1β, and TNF-α were measured in patients with pulmonary systolic arterial pressure (PAPs) >30 mm Hg and in patients with cirrhosis. In all, Six out of 11 patients with PAPs >30 mm Hg had PPHTN on right heart catheterization. The remaining 10 patients had an hyperdynamic circulation (HC). In PPHTN patients, ET-1 and IL-6 were significantly higher compared with HC and patients with cirrhosis. Endothelin 1 and IL-6 could be implicated in the pathogenesis of PPHTN. On the basis of these results, ET-1 receptor antagonists or anti-IL-6 could have a rationale in the treatment of PPHTN.

MeSH terms

  • Cardiac Catheterization
  • Cytokines / blood*
  • Echocardiography, Doppler, Color
  • Endothelin-1 / blood
  • Humans
  • Hypertension, Portal / blood*
  • Hypertension, Portal / complications
  • Hypertension, Portal / diagnostic imaging
  • Hypertension, Pulmonary / blood*
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / physiopathology
  • Interleukin-1beta / blood
  • Interleukin-6 / blood
  • Liver Cirrhosis / blood
  • Liver Cirrhosis / surgery*
  • Liver Transplantation*
  • Middle Aged
  • Tumor Necrosis Factor-alpha / blood


  • Cytokines
  • Endothelin-1
  • Interleukin-1beta
  • Interleukin-6
  • Tumor Necrosis Factor-alpha