Pulmonary-hepatic vascular disorders are frequent complications in patients with portal hypertension and cirrhosis. Both hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are associated with increased morbidity and mortality. The diagnosis of HPS should be confirmed early by arterial blood gas analysis and contrast enhanced echocardiography whereas POPH is finally diagnosed by presence of pulmonary arterial hypertension evaluated via right heart catheterization and presence of portal hypertension. Therapeutic options are initiation of long term oxygen therapy and liver transplantation in patients with severe HPS. Patients with POPH should receive targeted medical therapies with endothelin receptor antagonists, phosphodiesterase-5 inhibitors and/or prostanoids. In contrast, β-blockers should be avoided. It is unclear whether liver transplantation cures POPH or not. This review summarizes current knowledge of underlying conditions and focuses on therapeutic options in patients with pulmonary-hepatic vascular disorders.
