门脉性肺动脉高压的诊断与治疗进展

Progress in diagnosis and treatment of patients with portopulmonary hypertension

门脉性肺动脉高压是伴或不伴晚期肝病患者在门静脉高压基础上出现以肺动脉压升高和肺血管阻力增加为特点的疾病。门脉性肺动脉高压显著增加患者病死率。目前尚无明确有效的治疗药物,肝移植是唯一有效的治疗手段。本文重点介绍了门脉性肺动脉高压的流行病学、发病机制、临床表现、诊断、治疗及预后情况。

Portopulmonary hypertension （PPHTN） is defined as the development of pulmonary arterial hypertension associ-ated with increased pulmonary vascular resistance complicating portal hypertension, with or without advanced liver disease. The presence of PPHTN markedly increases mortality. No effective medical therapies are currently available and liver transplantation is the only accepted treatment at the time. This review stresses on the epidemiology, pathophysiology, clinical presentation, diagnosis, treatment and the prognosis of PPHTN.