title: Portopulmonary hypertension: survival and prognostic factors.
abstract: BACKGROUND
  Portopulmonary hypertension (PoPH) can be defined as elevation of pulmonary arterial pressure and pulmonary vascular resistance in the setting of portal hypertension. Survival results in PoPH are contrasting, and prognostic factors need to be identified.
  
  
  OBJECTIVE
  To analyze long-term survival in a large cohort of patients with PoPH with the aim of determining the independent variables affecting survival.
  
  
  METHODS
  We retrospectively analyzed charts of all patients referred to the French Referral Center for pulmonary arterial hypertension with the diagnosis of PoPH between 1984 and 2004.
  
  
  RESULTS
  The study population comprised 154 patients; 57% male. Mean age at diagnosis was 49 +/- 11 years, 60% of patients were in New York Heart Association functional class III-IV, and mean 6-minute walk distance was 326 +/- 116 m. Hemodynamic measurements showed a mean pulmonary arterial pressure of 53 +/- 13 mm Hg, cardiac index of 2.9 +/- 0.9 L/min/m(2), and pulmonary vascular resistance of 752 +/- 377 dyn/s/cm(5). Portal hypertension was related to cirrhosis in 136 patients, with a severity assessed as follows: Child-Pugh class A 51%, Child-Pugh class B 38%, Child-Pugh class C 11%. Overall survival rates at 1, 3, and 5 yr were 88, 75, and 68%, respectively. Multivariate regression analysis individualized the presence and severity of cirrhosis and cardiac index as major independent prognostic factors.
  
  
  CONCLUSIONS
  Prognosis in PoPH is mainly related to the presence and severity of cirrhosis and to cardiac function. The place of pulmonary arterial hypertension-specific therapies remains to be determined in the setting of PoPH.
authors.affiliation: Université Paris-Sud 11, UPRES 2705, Service de Pneumologie, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart, France. lepavec@gmail.com
authors.forename: Jérôme
authors.initials: J
authors.lastname: Le Pavec
authors.forename: Rogério
authors.initials: R
authors.lastname: Souza
authors.forename: Philippe
authors.initials: P
authors.lastname: Herve
authors.forename: Didier
authors.initials: D
authors.lastname: Lebrec
authors.forename: Laurent
authors.initials: L
authors.lastname: Savale
authors.forename: Colas
authors.initials: C
authors.lastname: Tcherakian
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Azzedine
authors.initials: A
authors.lastname: Yaïci
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
mesh_terms: D000328:Adult; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006975:Hypertension, Portal / Q000401:mortality* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery; D053208:Kaplan-Meier Estimate; D008103:Liver Cirrhosis / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D014655:Vascular Resistance; D016278:Ventricular Function, Right