Beyond Mutual Exclusivity: Coexistence and Sequential Evolution of Hepatopulmonary Syndrome and Portopulmonary Hypertension

Although hepatopulmonary syndrome and portopulmonary hypertension are traditionally viewed as opposing pulmonary vascular phenotypes of portal hypertension, one dominated by intrapulmonary vasodilatation and shunting, the other by pulmonary arterial remodeling and elevated pulmonary vascular resistance, published case reports and small case series demonstrate that they are not strictly mutually exclusive. Coexistence has been described, and sequential evolution has been reported in both directions. These observations suggest that shared but dynamically regulated vascular pathways may determine the dominant clinical phenotype over time.