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门肺高压症(portopulmonary hypertension,PPHTN)是在门静脉高压症(portal hyper tension,PHT)基础 上发生的以肺动脉压(pulmonary arlery pressure,PAP)升高(平静状态T>25 mmHg、活动状态T>30 mmHg)、肺血管阻力(pulmonary vascular resistance,PVR)增加(>240-250 dyn.sec.cm-5)而肺动脉楔(pulmonary arteriole wedge pressure.PAWP)正常(