Portopulmonary hypertension: A tale of two circulations

Rohit Budhiraja, Paul M. Hassoun

Research output: Contribution to journalReview articlepeer-review


Pulmonary involvement is common in patients with portal hypertension and can manifest in diverse manners. Changes in pulmonary arterial resistance, manifesting either as the hepatopulmonary syndrome or portopulmonary hypertension (PPHTN), have been increasingly recognized in these patients in recent years. This review summarizes the clinicopathologic features, diagnostic criteria, as well as the latest concepts in the pathogenesis and management of PPHTN, which is defined as an elevated pulmonary artery pressure in the setting of an increased pulmonary vascular resistance and a normal wedge pressure in a patient with portal hypertension.

Original languageEnglish (US)
Pages (from-to)562-576
Number of pages15
Issue number2
StatePublished - Feb 1 2003


  • Endothelin
  • Liver
  • Nitric oxide
  • Portal hypertension
  • Portopulmonary hypertension
  • Prostacyclin
  • Pulmonary hypertension
  • Serotonin
  • Transplant

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine


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