关键词: 门脉高压相关性肺动脉高压, 诊断, 治疗

Abstract: Portopulmonary hypertension (PoPH) represents one of complications occurred in patients with portal hypertension, which is characterized by high mortality rate and poor prognosis. The pathogenesis of PoPH remains unclear; it is believed to involve a complex interplay of factors including hyperdynamic circulation, imbalance of vasoactive substances, genetic factors and inflammatory responses. Up to the present, there is no specific treatment or medication for PoPH. However, medications such as endothelin receptor antagonists, prostacyclins and their analogues, phosphodiesterase-5 inhibitors and guanylate cyclase stimulants have been applied in the treatment of PoPH patients. The efficacy and safety of these treatment approaches still require further validation through large-scale, multicentered and randomized controlled trials. Liver transplantation may benefit a sub-group of patients but need a comprehensive assessment of individual cases. This article reviews the diagnosis, epidemiology, pathophysiology and current therapeutic performance of PoPH to further understand the pathogenesis of the disease and to improve diagnosis and treatment. Future research should focus on the development of new therapeutic drugs and evaluation of long-term effects of existing treatment methods, thereby providing more effective and safer treatment options to patients with PoPH.

Key words: portopulmonary hypertension, diagnosis, treatment