Thomas Christopher, Glinskii Vladimir, de Jesus Perez Vinicio, Sahay Sandeep
Division of Pulmonary, Allergy & Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, United States.
Houston Methodist Hospital Lung Center, Houston Methodist Hospital, Houston, TX, United States.
Front Med (Lausanne). 2020 Nov 3;7:569413. doi: 10.3389/fmed.2020.569413. eCollection 2020.
Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies.
门静脉高压性肺动脉高压（PoPH）被定义为与门静脉高压相关的肺动脉高压（PAH），是1类肺动脉高压（PH）的一个子集。PoPH是门静脉高压患者（无论有无肝病）发生严重发病和死亡的一个原因。在过去20年里，在阐明发病机制、有效的筛查算法、准确的诊断和治疗选择方面取得了重大进展。与特发性肺动脉高压（IPAH）和其他形式的PAH相比，PoPH患者的生存率仍然很低。最近，在这一患者群体中进行了第一项随机对照试验，结果显示PAH特异性治疗取得了有希望的结果。尽管对血流动力学和功能结局有积极影响，但尚不清楚PAH特异性治疗对长期生存或移植结局是否有有益作用。在本综述中，我们将讨论PoPH的流行病学、病理生理学、临床和血流动力学特征。此外，本综述将突出我们当前管理策略中的空白、面临的挑战，并为潜在有用的未来管理策略提供方向。
