title: Idiopathic portal hypertension: natural history and long-term outcome.
abstract: Idiopathic portal hypertension (IPH) is a rare cause of intrahepatic portal hypertension. Data on natural history and prognosis of IPH are limited. We sought to describe the complications and long-tem outcome of IPH by retrospectively studying 69 biopsy-proven cases of IPH. Mean duration of follow-up was 6.7 ± 4.6 years. All patients had evidence of portal hypertension (PH) at diagnosis, and 42% were symptomatic. Variceal bleeding (VB) was the most common manifestation. In those without bleeding at diagnosis, 74% had varices at first endoscopy. In those with large varices, the 1-year probability of first bleeding despite primary prophylaxis was 9%. The 1-year probability of rebleeding was 22%. Ascites and hepatic encephalopathy was documented in 26% and 7% of patients, respectively, at least once during the clinical course. The 1-year probability of developing portal vein thrombosis (PVT) was 9%, and 53% of patients receiving anticoagulation achieved recanalization. Human immunodeficiency virus (HIV) infection and VB at diagnosis were the independent predictors of PVT. Seven patients died (6 as a result of an IPH-related cause) and 2 were transplanted. Probability of liver transplantation-free survival was 82% at 10 years. Presence of a severe associated disorder and ascites as a presenting symptom were associated with poor survival.
  
  
  CONCLUSIONS
  Variceal bleeding is a major complication of IPH. Using, in IPH patients, the same management approach for PH as in cirrhosis is safe and maintains a low incidence of first bleeding and rebleeding in IPH patients. PVT is a frequent complication, particularly in those with HIV infection. Despite several complications, overall survival of patients with IPH is considerably good.
authors.affiliation: Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives i Metaboliques, Hospital Clínic-Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.
authors.forename: Sith
authors.initials: S
authors.lastname: Siramolpiwat
authors.forename: Susana
authors.initials: S
authors.lastname: Seijo
authors.forename: Rosa
authors.initials: R
authors.lastname: Miquel
authors.forename: Annalisa
authors.initials: A
authors.lastname: Berzigotti
authors.forename: Angeles
authors.initials: A
authors.lastname: Garcia-Criado
authors.forename: Anna
authors.initials: A
authors.lastname: Darnell
authors.forename: Fanny
authors.initials: F
authors.lastname: Turon
authors.forename: Virginia
authors.initials: V
authors.lastname: Hernandez-Gea
authors.forename: Jaume
authors.initials: J
authors.lastname: Bosch
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Garcia-Pagán
mesh_terms: D000328:Adult; D016145:Endoscopy, Digestive System; D005260:Female; D005500:Follow-Up Studies; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D008102:Liver Circulation; D008103:Liver Cirrhosis / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010198:Pancytopenia / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D013030:Spain / Q000453:epidemiology; D013163:Splenomegaly / Q000150:complications* / Q000401:mortality / Q000503:physiopathology*; D013927:Thrombosis / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult; D000094724:Idiopathic Noncirrhotic Portal Hypertension