Department of Internal Medicine, Division of Pulmonary and Critical Care, Mayo Clinic, Rochester, MN.
Chest. 2023 Jul;164(1):206-214. doi: 10.1016/j.chest.2023.01.009. Epub 2023 Jan 14.
Portopulmonary hypertension (POPH) affects 5% to 6% of patients with advanced liver disease and accounts for 5% to 15% of pulmonary arterial hypertension (PAH) cases. Compared with idiopathic PAH, POPH is associated with significantly worse survival. Recent studies have improved our understanding of the role of both PAH therapy and liver transplantation (LT) in the management of POPH and their impact on overall prognosis. We performed a review of the published literature to summarize the available evidence and guidelines regarding the diagnosis and management of POPH. POPH is defined by the presence of precapillary pulmonary hypertension in the context of portal hypertension. POPH is associated with increased perioperative risk at the time of LT, which can be stratified by mean pulmonary arterial pressure and pulmonary vascular resistance. Screening with echocardiography is recommended in all LT candidates to facilitate detection and treatment of POPH. Despite a paucity of evidence, POPH is treated similarly to idiopathic PAH with PAH therapy. These therapies are associated with improved pulmonary hemodynamics and facilitation of safe LT. LT can result in improvement or resolution of POPH in one-half of patients and has been associated with improved survival in highly selected patients. In summary, the prognosis in POPH is poor and is impacted by the severity of both pulmonary hypertension and liver disease. Management with a combination of PAH therapy and LT in selected patients has been associated with improved pulmonary hemodynamics and survival.
肝肺高血压（POPH）影响 5%至 6%的晚期肝病患者，占肺动脉高压（PAH）病例的 5%至 15%。与特发性 PAH 相比，POPH与明显更差的生存相关。最近的研究提高了我们对 PAH 治疗和肝移植（LT）在 POPH 管理中的作用及其对整体预后的影响的理解。我们对已发表的文献进行了回顾，以总结关于 POPH 的诊断和管理的现有证据和指南。POPH 定义为在门静脉高压的背景下存在毛细血管前肺动脉高压。POPH 与 LT 时围手术期风险增加有关，可通过平均肺动脉压和肺血管阻力分层。建议对所有 LT 候选者进行超声心动图筛查，以促进 POPH 的检测和治疗。尽管证据不足，但 POPH 的治疗类似于特发性 PAH，使用 PAH 治疗。这些治疗方法与改善肺血流动力学和促进安全 LT 有关。LT 可使一半患者的 POPH 改善或缓解，并与高度选择的患者的生存改善相关。总之，POPH 的预后较差，受肺动脉高压和肝病严重程度的影响。在选定的患者中，PAH 治疗和 LT 的联合管理与改善肺血流动力学和生存相关。
