title: Distinctive clinical features of portopulmonary hypertension.
abstract: OBJECTIVE
  To differentiate the cardiopulmonary profile of portopulmonary hypertension (PPHTN) from that of primary pulmonary hypertension and chronic liver disease.
  
  
  METHODS
  Retrospective survey.
  
  
  METHODS
  Tertiary care center.
  
  
  METHODS
  Thirty patients with cardiac catheterization-proven PPHTN were compared to 30 randomly selected patients with primary pulmonary hypertension alone and 30 patients with chronic liver disease alone necessitating consideration of liver transplantation (L-CONT).
  
  
  METHODS
  All patients underwent right heart catheterization, echocardiography, ECG, chest radiography, pulmonary function tests, ventilation-perfusion scanning, and room air arterial blood gas measurements.
  
  
  RESULTS
  Patients with PPHTN exhibited elevated pulmonary pressures (mean pulmonary pressure, 48.6+/-2.1 mm Hg) and pulmonary vascular resistance (11.6+/-1.6 mm Hg/L/min/m2) with simultaneous elevation in the cardiac index (3.8+/-0.3 L/min/m2) and depression of systemic vascular resistance (24.9+/-1.7 mm Hg/L/min/m2). Arterial blood gas measurements indicate that PPHTN exhibits a significant accentuation of the chronic respiratory alkalosis (PCO2, 28.7+/-0.5 mm Hg) usually seen with chronic liver disease and pulmonary hypertension. In addition, patients with PPHTN have an increased alveolar-arterial gradient (27.0+/-2.7 mm Hg) when compared to patients with L-CONT, suggesting impaired gas exchange.
  
  
  CONCLUSIONS
  PPHTN is associated with a unique clinical profile that possesses characteristics common to and exclusive of liver disease and primary pulmonary hypertension.
authors.affiliation: Department of Surgery, University of Maryland Medical System, Baltimore, USA. pkuo@surgery1.ab.umd.edu
authors.forename: P C
authors.initials: PC
authors.lastname: Kuo
authors.forename: J S
authors.initials: JS
authors.lastname: Plotkin
authors.forename: L B
authors.initials: LB
authors.lastname: Johnson
authors.forename: C D
authors.initials: CD
authors.lastname: Howell
authors.forename: J M
authors.initials: JM
authors.lastname: Laurin
authors.forename: S T
authors.initials: ST
authors.lastname: Bartlett
authors.forename: L J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000472:Alkalosis, Respiratory / Q000503:physiopathology; D001794:Blood Pressure / Q000502:physiology; D002245:Carbon Dioxide / Q000097:blood; D006328:Cardiac Catheterization; D002302:Cardiac Output / Q000502:physiology; D016022:Case-Control Studies; D002908:Chronic Disease; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006321:Heart / Q000503:physiopathology; D006801:Humans; D006975:Hypertension, Portal / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008107:Liver Diseases / Q000503:physiopathology; D016031:Liver Transplantation; D008168:Lung / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D011651:Pulmonary Artery / Q000503:physiopathology; D011659:Pulmonary Gas Exchange / Q000502:physiology; D011859:Radiography; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014655:Vascular Resistance / Q000502:physiology; D014692:Ventilation-Perfusion Ratio
chemical_list: D002245:Carbon Dioxide; D010100:Oxygen