Portopulmonary hypertension

Research output: Contribution to journalReview article

43 Scopus citations

Abstract

Portopulmonary hypertension (POPH) refers to the presence of pulmonary arterial hypertension (PAH) in patients with portal hypertension. Pulmonary hypertension in patients with liver disease or portal hypertension can be due to multiple mechanisms, including hyperdynamic (high-flow) state, increased pulmonary venous congestion, and vascular constriction or obstruction of the pulmonary arterial bed. Vascular obstruction to pulmonary arterial flow, reflected by increased pulmonary vascular resistance (PVR), is a key parameter that defines POPH. Among patients with portal hypertension, reported incidence rates of POPH range from 2 to 9%. Long-term survival in cases of POPH is poor. Favorable responses to pulmonary vasodilator/vasomodulatory therapy have been observed, but prospective, randomized trials are lacking. Severe POPH with right ventricular failure despite vasodilator therapy is associated with poor outcomes in the setting of liver transplantation (LT) and is considered a contraindication to LT. The post-LT course of patients with moderate POPH is unpredictable, but most patients can be weaned from PAH-specific therapy over time.

Original languageEnglish (US)
Pages (from-to)17-25
Number of pages9
JournalSeminars in Respiratory and Critical Care Medicine
Volume33
Issue number1
DOIs
StatePublished - Mar 23 2012

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Keywords

  • liver transplant
  • portal hypertension
  • portopulmonary hypertension
  • pulmonary hypertension

ASJC Scopus subject areas

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

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