Coexisting Hepatopulmonary Syndrome and Portopulmonary Hypertension

Implications for Liver Transplantation

Abstract

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) represent pulmonary complications of liver disease and portal hypertension. The underlying pathophysiology behind these entities is complex and involves different effects of vasoactive substances on the pulmonary vasculature, among them endothelin-1 and nitric oxide (NO). Hepatopulmonary syndrome results from vasodilation, intrapulmonary shunting, and hypoxia. In contrast, portopulmonary hypertension is predominantly owing to generalized vasoconstriction that leads to remodeling and an increase in pulmonary vascular resistance, but is rarely associated with hypoxia. We present a case report in which these 2 processes with opposing pathologic mechanisms coexist in the same patient. We also conducted a literature search to identify other documented cases of coexisting hepatopulmonary syndrome and portopulmonary hypertension, common clinical features of these patients, and outcomes with or without treatment. Our case highlights the importance of recognizing the coexistence of these 2 disease processes, as they may occur simultaneously and affect the approach to treatment, including liver transplantation.