Author information
1Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, ML 0564, Medical Sciences Building, 231 Albert Sabin Way, Cincinnati, OH 45267, USA. Electronic address: josean@ucmail.uc.edu.
2Department of Anesthesiology, University of Cincinnati, ML 3553, Medical Sciences Building, 231 Albert Sabin Way, Cincinnati, OH 45267, USA.
3Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, ML 0564, Medical Sciences Building, 231 Albert Sabin Way, Cincinnati, OH 45267, USA.
Abstract
Portopulmonary hypertension (PoPH) is a progressive, ultimately fatal cardiopulmonary disease that occurs exclusively in patients with underlying portal hypertensive liver disease. PoPH outcomes are driven by both the severity of underlying liver disease and the degree of cardiac adaptation to elevated pulmonary pressures. The mainstay of treatment in PoPH is targeted pulmonary vascular therapy. Liver transplantation (LT) can be beneficial in some patients, but is associated with considerable risks in the PoPH population, and outcomes are variable. The optimal management strategy for PoPH, LT, or medical therapy alone, is unclear, and further research is needed to help guide clinical decision-making.