Department of Medicine, M.S., Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.
Am J Respir Crit Care Med. 2013 Jan 15;187(2):133-43. doi: 10.1164/rccm.201209-1583CI. Epub 2012 Nov 15.
Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients.
肝肺综合征和门肺高压是两种肝脏疾病相关的肺血管并发症。两种疾病的病理生理学基础不同，但任何一种疾病的患者都可能会出现呼吸困难。仔细评估伴随症状、体格检查、肺功能检查和动脉血气分析，以及超声心动图、影像学和血流动力学研究，对于确立（和区分）这些诊断至关重要。在过去十年中，我们对这些疾病的病理生物学、自然史和治疗方法的理解有了显著的提高；然而，这些疾病的存在仍然会增加基础肝病患者的发病率和死亡率。肝肺综合征目前尚无有效的药物治疗方法。虽然肝移植可以治愈肝肺综合征，但即使进行了肝移植，患者的生存率似乎也更差。对于存在严重门肺高压的患者，肝移植风险极高，而靶向药物治疗可以改善其功能状态，并使少数选择的患者能够成功接受移植。
