title: Effectiveness of phosphodiesterase-5 inhibitor therapy for portopulmonary hypertension.
abstract: BACKGROUND
  Portopulmonary hypertension is associated with significant morbidity and mortality. Phosphodiesterase-5 inhibitor therapy is efficacious in other causes of WHO group I pulmonary arterial hypertension.
  
  
  OBJECTIVE
  To evaluate the efficacy and safety of phosphodiesterase-5 inhibitor therapy in patients with portopulmonary hypertension.
  
  
  METHODS
  A single-centre retrospective cohort study that included patients with a diagnosis of portopulmonary hypertension was performed. The primary outcome was change in pulmonary vascular resistance after six months of phosphodiesterase-5 inhibitor therapy. A secondary evaluation investigated the effect on other hemodynamic measurements, 6 min walk distance, functional class, safety outcomes and survival.
  
  
  RESULTS
  Of 1385 patients screened, 25 patients with portopulmonary hypertension were identified, of whom 20 received a phosphodiesterase-5 inhibitor. After six months, there was a significant decrease in pulmonary vascular resistance (-236 dyn • s • cm(-5) [95% CI -343 dyn • s • cm(-5) to -130 dyn • s • cm(-5)]; P<0.001), mean pulmonary artery pressure (-8.9 mmHg [95% CI -13.7 mmHg to -4.2 mmHg]; P=0.001) and an increase in Fick cardiac output (0.9 L/min [95% CI 0.1 L/min to 1.6 L/min]; P=0.02). There was no change in 6 min walk distance. The proportion of subjects with a WHO functional class III or IV was significantly reduced at six months compared with baseline (18% versus 61%; P=0.002). Safety outcomes did not reveal any adverse events.
  
  
  CONCLUSIONS
  Phosphodiesterase-5 inhibitor therapy improved hemodynamics and functional class at six months in a cohort of patients with portopulmonary hypertension.
authors.forename: Jolene H
authors.initials: JH
authors.lastname: Fisher
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
authors.forename: Cathy
authors.initials: C
authors.lastname: Chau
authors.forename: Amie T
authors.initials: AT
authors.lastname: Kron
authors.forename: John T
authors.initials: JT
authors.lastname: Granton
mesh_terms: D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy*; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D058986:Phosphodiesterase 5 Inhibitors