Department of Medicine, Emory University, Atlanta, GA 30322, USA.
J Clin Gastroenterol. 2010 Aug;44(7):e136-40. doi: 10.1097/MCG.0b013e3181da76fc.
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) represent pulmonary complications of liver disease and portal hypertension. The underlying pathophysiology behind these entities is complex and involves different effects of vasoactive substances on the pulmonary vasculature, among them endothelin-1 and nitric oxide (NO). Hepatopulmonary syndrome results from vasodilation, intrapulmonary shunting, and hypoxia. In contrast, portopulmonary hypertension is predominantly owing to generalized vasoconstriction that leads to remodeling and an increase in pulmonary vascular resistance, but is rarely associated with hypoxia. We present a case report in which these 2 processes with opposing pathologic mechanisms coexist in the same patient. We also conducted a literature search to identify other documented cases of coexisting hepatopulmonary syndrome and portopulmonary hypertension, common clinical features of these patients, and outcomes with or without treatment. Our case highlights the importance of recognizing the coexistence of these 2 disease processes, as they may occur simultaneously and affect the approach to treatment, including liver transplantation.
肝肺综合征（HPS）和门肺高压（POPH）代表了肝脏疾病和门静脉高压的肺部并发症。这些疾病背后的病理生理学非常复杂，涉及到血管活性物质对肺血管的不同影响，其中包括内皮素-1 和一氧化氮（NO）。肝肺综合征是由于血管扩张、肺内分流和缺氧引起的。相比之下，门肺高压主要是由于全身性血管收缩导致肺血管阻力增加和重塑引起的，但很少与缺氧有关。我们报告了一个病例，其中这两种具有相反病理机制的过程共存于同一患者体内。我们还进行了文献检索，以确定其他共存的肝肺综合征和门肺高压的病例，这些患者的共同临床特征，以及治疗或不治疗的结果。我们的病例强调了认识到这两种疾病过程同时存在的重要性，因为它们可能同时发生，并影响治疗方法，包括肝移植。
