抄録
Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension in patients with or without cirrhosis, and it results from complex pathophysiological interactions between the portal and pulmonary circulations. PoPH is a relatively rare complication of portal hypertension, and female sex, autoimmune hepatitis as the underlying etiology, and portosystemic shunt development have been reported as clinical risk factors for PoPH. The prognosis of this disease is quite poor, which could be related to the delayed diagnosis and treatment of PoPH compared with PAH caused by other etiologies. In clinical practice, echocardiography, with which we can noninvasively estimate pulmonary arterial pressure, is used as a gatekeeper for the diagnosis of PAH including PoPH prior to right heart catheterization, which is the gold standard for the establishment of a definite diagnosis of pulmonary hypertension. Recently, multidrug therapies including endothelin receptor antagonists have come into commom use for patients with PoPH, providing good outcomes, and pharmacotherapies followed by liver transplantation are thought to be particularly appropriate for those with moderate to severe PoPH. In this review article, we summarize the current topics in the diagnosis and treatment of PoPH. In addition, we present our novel clinical research on this rare disease with a poor prognosis, along with an illustrative case.
