Hepatopulmonary syndrome and portopulmonary hypertension: implications for liver transplantation

Abstract

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are uncommon pulmonary vascular consequences of advanced liver disease. HPS, characterized by arterial hypoxemia caused by pulmonary vascular dilatation, may resolve completely after liver transplantation. POPH, caused by vasoproliferation/constriction and obstruction of pulmonary arterial blood flow, is associated with higher risk for liver transplantation and increased post-transplantation mortality. With or without transplantation, survival in patients who have these syndromes is associated with specific oxygenation and hemodynamic variables.