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This chapter focuses on two cardiopulmonary vascular disorders of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension. HPS is characterized by gas exchange disturbances due to pulmonary vasodilation and right-to-left shunting, whereas portopulmonary hypertension is defined by pulmonary hypertension secondary to increased pulmonary vascular resistance, which may lead to hemodynamic failure. Key aspects of assessment include active screening with echocardiography, right heart catheterization, and arterial blood gas analysis, early diagnosis, and prompt referral to specialized hepatology centers. As no curative medical treatment is available and only liver transplantation may lead to resolution, MELD exceptions can be allocated to provide prioritization for transplantation. This chapter will give an overview on diagnosis and screening, pathophysiology and pathogenesis, therapeutic targets, and treatment of both conditions. |
