title: Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.
abstract: BACKGROUND
  Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service.
  
  
  METHODS
  A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010.
  
  
  RESULTS
  Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, with a balanced distribution in gender. Alcohol (n = 57) and hepatitis C (n = 10) were the most common causes of portal hypertension. Phosphodiesterase V inhibitors were the most frequently used targeted therapy, in 63.6% (n = 70) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, or year of diagnosis on survival.
  
  
  CONCLUSIONS
  Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: Sasiharan
authors.initials: S
authors.lastname: Sithamparanathan
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.
authors.forename: Arun
authors.initials: A
authors.lastname: Nair
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: Logan
authors.initials: L
authors.lastname: Thirugnanasothy
authors.affiliation: Pulmonary Hypertension Unit, Royal Free Hospital, London, United Kingdom.
authors.forename: J Gerry
authors.initials: JG
authors.lastname: Coghlan
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
authors.forename: Robin
authors.initials: R
authors.lastname: Condliffe
authors.affiliation: Royal Brompton Pulmonary Hypertension and Adult Congenital Heart Centre, Imperial College, London, United Kingdom.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
authors.forename: Charlie A
authors.initials: CA
authors.lastname: Elliot
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Fisher
authors.affiliation: Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
authors.forename: Sean
authors.initials: S
authors.lastname: Gaine
authors.affiliation: National Heart and Lung Institute, Imperial College, London and Hammersmith Hospital, London, United Kingdom.
authors.forename: J Simon R
authors.initials: JSR
authors.lastname: Gibbs
authors.affiliation: Royal Brompton Pulmonary Hypertension and Adult Congenital Heart Centre, Imperial College, London, United Kingdom.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: Pulmonary Hypertension Unit, Royal Free Hospital, London, United Kingdom.
authors.forename: Clive
authors.initials: C
authors.lastname: E Handler
authors.affiliation: National Heart and Lung Institute, Imperial College, London and Hammersmith Hospital, London, United Kingdom.
authors.forename: Luke S
authors.initials: LS
authors.lastname: Howard
authors.affiliation: Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, Scotland.
authors.forename: Martin
authors.initials: M
authors.lastname: Johnson
authors.affiliation: Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
authors.forename: David G
authors.initials: DG
authors.lastname: Kiely
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.
authors.forename: James L
authors.initials: JL
authors.lastname: Lordan
authors.affiliation: Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, Scotland.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Peacock
authors.affiliation: Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom.
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.affiliation: Pulmonary Hypertension Unit, Royal Free Hospital, London, United Kingdom.
authors.forename: Benjamin E
authors.initials: BE
authors.lastname: Schreiber
authors.affiliation: Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom.
authors.forename: Karen K K
authors.initials: KKK
authors.lastname: Sheares
authors.affiliation: Royal Brompton Pulmonary Hypertension and Adult Congenital Heart Centre, Imperial College, London, United Kingdom.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Wort
authors.affiliation: National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom. Electronic address: paul.corris@ncl.ac.uk.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Corris
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000401:mortality* / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult
keywords: PAH-targeted treatment; portal hypertension; portopulmonary hypertension; pulmonary arterial hypertension; survival