Ioachimescu O C, Mehta A C, Stoller J K
Cleveland Clinic, Department of Sleep Medicine, 9500 Euclid Ave FA20, Cleveland, OH 44195, USA.
Eur Respir J. 2007 Jun;29(6):1277-80. doi: 10.1183/09031936.00140306.
Portopulmonary hypertension (PPHTN) and hepatopulmonary syndrome (HPS) are distinct clinical entities that may accompany liver disease. While PPHTN and HPS have been infrequently described as occurring in the same patient, to the present authors' knowledge, the order of occurrence has always been the initial onset of HPS, with pulmonary hypertension developing either concurrently or subsequently. In some instances, liver transplantation has been undertaken for HPS, followed by resolution of the HPS and subsequent development of pulmonary hypertension. The current case study presents a patient with hepatitis C-related cirrhosis in whom PPTHN developed initially, followed 2 yrs later by the development of the HPS. The current authors speculate that progressive imbalance in favour of endogenous vasodilators over vasoconstrictive factors led to normalisation of the pulmonary artery pressures.
门肺高压（PPHTN）和肝肺综合征（HPS）是可能伴随肝脏疾病出现的不同临床实体。虽然PPHTN和HPS很少被描述为在同一患者中发生，但据作者所知，发生顺序一直是HPS首先出现，随后肺动脉高压同时或相继出现。在某些情况下，已针对HPS进行肝移植，随后HPS得到缓解，继而出现肺动脉高压。本病例研究介绍了一名丙型肝炎相关性肝硬化患者，该患者最初发生了PPTHN，2年后出现了HPS。作者推测，内源性血管舒张因子相对于血管收缩因子的渐进性失衡导致了肺动脉压正常化。
