Salzano Andrea, Sirico Domenico, Golia Luisa, Faga Valentina, Flora Martina, Bossone Eduardo, Cittadini Antonio
Department of Translational Medical Sciences, University "Federico II", Naples, Italy.
Department of Cardiology and Cardiac Surgery, University Hospital "Scuola Medica Salernitana", Salerno, Italy.
Monaldi Arch Chest Dis. 2013 Jun;80(2):66-8. doi: 10.4081/monaldi.2013.81.
Portopulmonary hypertension (POPH) refers to the condition of pulmonary arterial hypertension (PAH) in association with portal hypertension. Among patients with portal hypertension, reported incidence rates of POPH range from 2 to 10% and long-term prognosis in cases of POPH is poor. Fundamental issues of management of POPH include identification of patients likely to benefit from liver transplantation (LTx) and treatment with specific pulmonary vasodilators before and after LTx. This review presents as an overview of the current knowledge on the epidemiology, diagnosis, treatment and prognosis of patients with portopulmonary hypertension.
门肺高压（POPH）是指与门静脉高压相关的肺动脉高压（PAH）状态。在门静脉高压患者中，报道的门肺高压发病率为2%至10%，且门肺高压患者的长期预后较差。门肺高压管理的基本问题包括识别可能从肝移植（LTx）中获益的患者，以及在肝移植前后使用特定的肺血管扩张剂进行治疗。本综述概述了目前关于门肺高压患者的流行病学、诊断、治疗和预后的知识。
