What's new in the treatment of portopulmonary hypertension?

Hilary M. Dubrock, Richard N. Channick, Michael Joseph Krowka

Research output: Contribution to journalArticle

16 Scopus citations

Abstract

Portopulmonary hypertension (POPH) is a complication of portal hypertension characterized by pulmonary vasoconstriction and vascular remodeling that can lead to right heart failure and death. Differentiation of POPH from other causes of pulmonary hypertension, such as volume overload or a hyperdynamic high flow state, is critical because a diagnosis of POPH has significant implications for liver transplant risk stratification, Model for End Stage Liver Disease exception points, and the use of pulmonary arterial hypertension-(PAH) specific therapy. Currently, there are 12 approved medications for the treatment of PAH in the US, and three of these were approved in 2013. This review will discuss the diagnosis, evaluation and management of POPH and the role of recently approved PAH therapies in the treatment of POPH.

Original languageEnglish (US)
Pages (from-to)983-992
Number of pages10
JournalExpert Review of Gastroenterology and Hepatology
Volume9
Issue number7
DOIs
StatePublished - Sep 1 2015

Keywords

  • macitentan
  • portopulmonary hypertension
  • pulmonary arterial hypertension
  • riociguat
  • treatment
  • treprostinil

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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