Clinical management of portopulmonary hypertension

Nga Lei Tam, Xiaoshun He

Research output: Contribution to journalReview articlepeer-review

19 Scopus citations


Background: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. Data sources: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. Results: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. Conclusions: After an accurate diagnosis of PPH, treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.

Original languageEnglish (US)
Pages (from-to)464-469
Number of pages6
JournalHepatobiliary and Pancreatic Diseases International
Issue number5
StatePublished - Oct 1 2007


  • Current development
  • Diagnosis
  • Pathogenesis
  • Portopulmonary hypertension
  • Therapy

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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