Update on Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a common pulmonary vascular complication of liver disease associated with significant morbidity and mortality. Liver transplant (LT) is frequently curative in these patients, but effective medical therapies remain elusive. The purpose of this review is to highlight key clinical features and recent updates in the management of HPS in order to empower clinicians to take optimal care of these patients.

Recent HPS studies have focused on outcomes in patients with HPS who undergo LT, perioperative management of HPS, and variables associated with prognosis. Animal studies have provided recent insight into disease pathogenesis, but effective medical therapy for HPS remains an active area of investigation.

Multiple studies have reaffirmed that patients with HPS who undergo LT have favorable outcomes. Beyond pre-LT PaO2, new research has demonstrated the importance of factors like degree of orthodeoxia in HPS prognostication. No medical therapy has been proven effective to date.