Nacif Lucas Souto, Andraus Wellington, Pinheiro Rafael Soares, Ducatti Liliana, Haddad Luciana B P, D'Albuquerque Luiz Carneiro
Department of Gastroenterology, Liver and Gastrointestinal Transplant Division, University of São Paulo, São Paulo, SP, Brazil.
Arq Bras Cir Dig. 2014 Apr-Jun;27(2):145-7. doi: 10.1590/s0102-67202014000200012.
The hepatopulmonary syndrome has been acknowledged as an important vascular complication in lungs developing systemic hypoxemia in patients with cirrhosis and portal hypertension. Is formed by arterial oxygenation abnormalities induced from intrapulmonary vascular dilatations with liver disease. It is present in 4-32% of patients with cirrhosis. It increases mortality in the setting of cirrhosis and may influence the frequency and severity. Initially the hypoxemia responds to low-flow supplemental oxygen, but over time, the need for oxygen supplementation is necessary. The liver transplantation is the only effective therapeutic option for its resolution.
To update clinical manifestation, diagnosis and treatment of this entity.
A literature review was performed on management of hepatopulmonary syndrome. The electronic search was held of the Medline-PubMed, in English crossing the headings "hepatopulmonary syndrome", "liver transplantation" and "surgery". The search was completed in September 2013.
Hepatopulmonary syndrome is classically defined by a widened alveolar-arterial oxygen gradient (AaPO2) on room air (>15 mmHg, or >20 mmHg in patients >64 years of age) with or without hypoxemia resulting from intrapulmonary vasodilatation in the presence of hepatic dysfunction or portal hypertension. Clinical manifestation, diagnosis, classification, treatments and outcomes are varied.
The severity of hepatopulmonary syndrome is an important survival predictor and determine the improvement, the time and risks for liver transplantation. The liver transplantation still remains the only effective therapeutic.
肝肺综合征已被公认为肝硬化和门静脉高压患者肺部发生系统性低氧血症的一种重要血管并发症。它由肝病导致的肺内血管扩张引起的动脉氧合异常所形成。肝硬化患者中4% - 32%存在该综合征。它会增加肝硬化患者的死亡率，并可能影响其发生频率和严重程度。起初，低氧血症对低流量补充氧气有反应，但随着时间推移，补充氧气变得必要。肝移植是解决该问题的唯一有效治疗选择。
更新该疾病的临床表现、诊断和治疗方法。
对肝肺综合征的管理进行文献综述。在Medline-PubMed上进行电子检索，检索词为英文，包括“肝肺综合征”“肝移植”和“手术”。检索于2013年9月完成。
肝肺综合征的经典定义为在静息状态下肺泡-动脉氧分压差（AaPO2）增大（>15 mmHg，64岁以上患者>20 mmHg），无论是否存在因肝功能不全或门静脉高压导致的肺内血管扩张引起的低氧血症。其临床表现、诊断、分类、治疗及预后各不相同。
肝肺综合征的严重程度是重要的生存预测指标，决定肝移植的改善情况、时机和风险。肝移植仍然是唯一有效的治疗方法。
