Distinctive clinical features of portopulmonary hypertension

Chest. 1997 Oct;112(4):980-6. doi: 10.1378/chest.112.4.980.


Study objective: To differentiate the cardiopulmonary profile of portopulmonary hypertension (PPHTN) from that of primary pulmonary hypertension and chronic liver disease.

Design: Retrospective survey.

Setting: Tertiary care center.

Patients: 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).

Interventions: 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.

Publication types

  • Comparative Study
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Alkalosis, Respiratory / physiopathology
  • Blood Pressure / physiology
  • Carbon Dioxide / blood
  • Cardiac Catheterization
  • Cardiac Output / physiology
  • Case-Control Studies
  • Chronic Disease
  • Echocardiography
  • Electrocardiography
  • Female
  • Heart / physiopathology
  • Humans
  • Hypertension, Portal / diagnostic imaging
  • Hypertension, Portal / physiopathology*
  • Hypertension, Pulmonary / diagnostic imaging
  • Hypertension, Pulmonary / physiopathology*
  • Liver Diseases / physiopathology
  • Liver Transplantation
  • Lung / physiopathology
  • Male
  • Middle Aged
  • Oxygen / blood
  • Pulmonary Artery / physiopathology
  • Pulmonary Gas Exchange / physiology
  • Radiography
  • Respiratory Function Tests
  • Retrospective Studies
  • Vascular Resistance / physiology
  • Ventilation-Perfusion Ratio


  • Carbon Dioxide
  • Oxygen