title: Phosphodiesterase 5 inhibitors lower both portal and pulmonary pressure in portopulmonary hypertension: a case report.
abstract: BACKGROUND
  Portopulmonary hypertension (PPHTN) is a severe complication in liver cirrhosis. PDE5 inhibitors lower pulmonary arterial pressure (PAP) in PPHTN. However, their effect on portal hypertension has not yet been investigated.
  
  
  METHODS
  A 55 year old male patient presented with PPHTN and alcoholic liver cirrhosis. 10 mg of Tadalafil, a PDE5 inhibitor with a long half-life, was administered orally under continuous monitoring of pulmonary and portal hemodynamics. For maintenance therapy the patient received Sildenafil 20 mg bid.Tadalafil lowered mean PAP from 45 to 39 mmHg within 60 minutes. Cardiac output (CO) increased from 6.8 to 7.9 l/min. Central venous pressure (CVP) remained stable at 3 mmHg. Systolic and diastolic blood pressure was lowered from 167/89 to 159/86 mmHg. Pulse rate increased from 75 to 87 per min. Wedged hepatic vein pressure (WHVP) decreased from 21 to 18 mm Hg, hepatovenous pressure gradient (HVPG) decreased from 10 to 7 mmHg. Hemodynamic monitoring after 6 months of Sildenafil therapy revealed a sustained lowering of mean PAP. HVPG remained constant at 10 mmHg. Cardiac and pulmonary performance had further improved.
  
  
  CONCLUSIONS
  This case report shows for the first time, that phosphodiesterase 5 inhibitors lower both portal and pulmonary pressure in portopulmonary hypertension.
authors.affiliation: Department of Gastroenterology, Hepatology, Endocrinology and Infectious Diseases, University Hospital, Freiburg, Germany. wolfgang.kreisel@uniklinik-freiburg.de.
authors.forename: Hinrich C
authors.initials: HC
authors.lastname: Bremer
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Kreisel
authors.forename: Kai
authors.initials: K
authors.lastname: Roecker
authors.forename: Michael
authors.initials: M
authors.lastname: Dreher
authors.forename: Daniel
authors.initials: D
authors.lastname: Koenig
authors.forename: Anna Katharina
authors.initials: AK
authors.lastname: Kurz-Schmieg
authors.forename: Hubert E
authors.initials: HE
authors.lastname: Blum
authors.forename: Martin
authors.initials: M
authors.lastname: Roessle
authors.forename: Peter
authors.initials: P
authors.lastname: Deibert