Current Approach to the Diagnosis and Management of Portopulmonary Hypertension

Lynn A. Fussner, Michael Joseph Krowka

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

Portopulmonary hypertension (POPH) is a form of pulmonary arterial hypertension occurring in the setting of portal hypertension with or without hepatic cirrhosis. The presence of both portal and pulmonary vascular disease contributes to complicated hemodynamics and therapeutic challenges, though the severities do not appear to correlate directly. Diagnosis of POPH, and distinction from the commonly observed hyperdynamic state of end-stage liver disease, is typically accomplished with an initial screening transthoracic echocardiogram, followed by right heart catheterization for confirmation of hemodynamic parameters. Though few studies have directly evaluated use in POPH, pulmonary artery-directed therapy is the cornerstone of management, along with consideration of liver transplantation. Perioperative and long-term outcomes are variable, but uniformly worse in the setting of uncontrolled pulmonary pressures. Risk stratification and optimal patient selection for these interventions are areas of ongoing investigation.

Original languageEnglish (US)
Article number29
JournalCurrent Gastroenterology Reports
Volume18
Issue number6
DOIs
StatePublished - Jun 1 2016

Keywords

  • Echocardiography
  • Liver transplant
  • Portopulmonary hypertension
  • Pulmonary arterial hypertension
  • Right heart catheterization
  • Vasodilator

ASJC Scopus subject areas

  • Gastroenterology

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