Successful switch from inhalative iloprost to oral bosentan in portopulmonary hypertension associated with liver cirrhosis

Wien Klin Wochenschr. 2004 Sep 30;116(17-18):627-30. doi: 10.1007/s00508-004-0238-2.


Portopulmonary hypertension (PPHTN) is a rare complication of liver cirrhosis. Prostanoids have been shown to be effective in the treatment of PPHTN and have been used as a bridge to orthotopic liver transplantation. However, inhibition of platelet aggregation might be a limitation of prostacyclin therapy in patients with end-stage liver disease having an increased risk of bleeding from esophageal varices. The effect of oral bosentan, a dual endothelin-receptor antagonist in the reversal of PPHTN, is still unclear. We report a case of PPHTN (mean pulmonary artery pressure [mPAP] of 51 mmHg) that was successfully switched from inhalative iloprost to oral bosentan therapy. Hemodynamic and symptomatic improvements were maintained after a 12-month long-term treatment with inhalative iloprost as well as after single oral bosentan therapy. This is the first reported case of a successful switch from therapy with an inhalative prostacyclin analogue to oral bosentan in a patient suffering from PPHTN. Thus, oral bosentan therapy might be a promising new option for patients suffering from PPHTN.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Administration, Inhalation
  • Administration, Oral
  • Antihypertensive Agents / administration & dosage*
  • Bosentan
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Hemodynamics
  • Humans
  • Hypertension, Portal / drug therapy*
  • Hypertension, Portal / etiology
  • Hypertension, Portal / physiopathology
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Iloprost / administration & dosage*
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / physiopathology
  • Liver Transplantation
  • Middle Aged
  • Platelet Aggregation Inhibitors / administration & dosage*
  • Sulfonamides / administration & dosage*
  • Time Factors


  • Antihypertensive Agents
  • Platelet Aggregation Inhibitors
  • Sulfonamides
  • Iloprost
  • Bosentan