title: Clinical management of portopulmonary hypertension.
abstract: BACKGROUND
  Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH.
  
  
  METHODS
  An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension.
  
  
  RESULTS
  Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH.
  
  
  CONCLUSIONS
  After an accurate diagnosis of PPH, treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.
authors: Organ Transplantation Center, First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China. ngalei@gmail.com
authors: Nga Lei
authors: NL
authors: Tam
authors: Xiao-Shun
authors: XS
authors: He
mesh_terms: D006328:Cardiac Catheterization; D004452:Echocardiography; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods*; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D010975:Platelet Aggregation Inhibitors; D014665:Vasodilator Agents