ABSTRACT

Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are pulmonary vascular complications of portal hypertension with or without cirrhosis. Although these two conditions may both initially present with dyspnea and are pathologically linked by the presence of portal hypertension, their pathophysiologic mechanisms, and hence their management and therapeutic approach, are significantly different. HPS is characterized by low pulmonary vascular resistance secondary to intrapulmonary vascular dilatations, and hypoxemia; POPH features elevated pulmonary vascular resistance due to obstruction of pulmonary arterial flow. This distinction is important if liver transplant (LT) is being considered due to differences in risk, treatment options, and outcomes. HPS is described in detail in Chapter 63 of this book.