title: Successful use of continuous intravenous prostacyclin in a patient with severe portopulmonary hypertension.
abstract: BACKGROUND
  Portopulmonary hypertension, defined by a mean pulmonary artery pressure > 25 mm Hg in the presence of normal pulmonary capillary wedge pressure and portal hypertension, is a known complication of end-stage liver disease that has been associated with high morbidity and mortality at the time of liver transplantation.
  
  
  METHODS
  Descriptive case report.
  
  
  METHODS
  A 32 year old male patient suffering from end-stage hepatitis C liver cirrhosis presented with severe portopulmonary hypertension. At presentation the following pulmonary hemodynamics were measured: systolic pulmonary artery pressure (PAP) 76 mm Hg, mean PAP 42 mm Hg, pulmonary vascular resistance index (PVRI) 931, pulmonary capillary wedge pressure (PCWP) 9 mm Hg, and cardiac output (CO) 4.03 l/min.
  
  
  METHODS
  After acute hemodynamic testing the patient received 8 ng/kg/min epoprostenol (prostacyclin) by continuous intravenous infusion with an infusion pump. Hemodynamic evaluation was performed monthly by transthoracic echocardiography and right heart catheterisation after 5 months.
  
  
  RESULTS
  After 5 months of continuous therapy right heart catheterisation revealed the following hemodynamics: systolic pulmonary artery pressure (PAP) 59 mm Hg, mean PAP 32 mm Hg, pulmonary vascular resistance index (PVRI) 561, pulmonary capillary wedge pressure (PCWP) 7 mm Hg, and cardiac output (CO) 6.95 l/min. This presents a decrease in systolic pulmonary artery pressure of approximately 22%, a decrease in mean pulmonary artery pressure of approximately 30%, a decrease in pulmonary vascular resistance of approximately 40% and an increase in cardiac output of approximately 73%. Echocardiography demonstrated a decrease in estimated systolic pulmonary artery pressure of about 37% after 8 months of therapy. No complications were observed during epoprostenol therapy.
  
  
  CONCLUSIONS
  In this adult patient suffering from end-stage liver disease and portopulmonary hypertension, administration of continuous intravenous epoprostenol resulted in significant reduction of pulmonary hypertension and therefore in acceptance for orthotopic liver transplantation. Utilisation of this new therapeutic strategy might be a helpful pharmacological tool for patients with portopulmonary hypertension to make them acceptable for orthotopic liver transplantation.
authors.affiliation: Division of General Internal Medicine-Pneumology Service, Faculty of Medicine, University of Innsbruck. Austria.CM.Kaehler@uibk.ac.at
authors.forename: C M
authors.initials: CM
authors.lastname: Kähler
authors.forename: I
authors.initials: I
authors.lastname: Graziadei
authors.forename: C J
authors.initials: CJ
authors.lastname: Wiedermann
authors.forename: M P
authors.initials: MP
authors.lastname: Kneussl
authors.forename: W
authors.initials: W
authors.lastname: Vogel
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D011464:Epoprostenol / Q000008:administration & dosage*; D019698:Hepatitis C, Chronic / Q000150:complications; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D007262:Infusions, Intravenous; D008103:Liver Cirrhosis / Q000150:complications* / Q000821:virology; D016031:Liver Transplantation; D008297:Male; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol