title: Portopulmonary hypertension: a report from the US-based REVEAL Registry.
abstract: BACKGROUND
  We evaluated survival and hospitalization rates in patients with group 1 portopulmonary hypertension (PoPH) in the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry).
  
  
  METHODS
  The REVEAL Registry is a multicenter, observational, US-based study evaluating demographics and management of patients with pulmonary arterial hypertension (PAH). Outcomes were examined using Kaplan-Meier time-to-event estimates and compared with patients with idiopathic PAH (IPAH) or familial PAH (FPAH).
  
  
  RESULTS
  One hundred seventy-four patients with PoPH were enrolled in the REVEAL Registry (IPAH/FPAH; n = 1,478) from March 2006 to December 2009. Mean age was 53 ± 10 years, 52% were female, 32% were newly diagnosed, and 6% were New York Heart Association/World Health Organization functional class IV. Outcome parameters were worse for PoPH vs IPAH/FPAH, respectively: 2-year survival from enrollment (67% vs 85%, P < .001), 5-year survival from time of diagnosis (40% vs 64%, P < .001), and 2-year freedom from all-cause hospitalization (49% vs 59%, P = .019). However, despite worse outcomes, hemodynamic parameters at diagnosis were better for PoPH vs IPAH/FPAH, respectively: mean pulmonary artery pressure (49 mm Hg vs 53 mm Hg, P < .001), mean right atrial pressure (9 mm Hg vs 10 mm Hg, P = .005), pulmonary vascular resistance (8 Wood units vs 12 Wood units, P < .001), and cardiac output (5 L/min vs 4 L/min, P < .001). Compared with patients with IPAH/FPAH, patients with PoPH were less likely to be on a PAH-specific therapy at enrollment (P < .001), suggesting potential delays in therapy for patients with PoPH.
  
  
  CONCLUSIONS
  Patients with PoPH had significantly poorer survival and all-cause hospitalization rates compared with patients with IPAH/FPAH, despite having better hemodynamics at diagnosis. Further studies should investigate such outcomes and differences in treatment patterns.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
authors.affiliation: Mayo Clinic, Rochester, MN. Electronic address: krowka.michael@mayo.edu.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Krowka
authors.affiliation: ICON Late Phase and Outcomes Research, San Francisco, CA.
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.affiliation: Columbia University College of Physicians and Surgeons, New York, NY.
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.affiliation: University of Pennsylvania Medical Center, Philadelphia, PA.
authors.forename: Darren
authors.initials: D
authors.lastname: Taichman
authors.affiliation: Cleveland Clinic, Cleveland, OH.
authors.forename: Raed A
authors.initials: RA
authors.lastname: Dweik
authors.affiliation: University of Colorado, Denver, CO.
authors.forename: David B
authors.initials: DB
authors.lastname: Badesch
authors.affiliation: Mayo Clinic, Rochester, MN.
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
mesh_terms: D005260:Female; D006439:Hemodynamics; D006760:Hospitalization; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D008107:Liver Diseases / Q000150:complications; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012042:Registries; D014481:United States