Cuadrado Antonio, Díaz Ainhoa, Iruzubieta Paula, Salcines José Ramón, Crespo Javier
Servicio de Aparato Digestivo, Hospital Universitario Marqués de Valdecilla, Santander, España.
Servicio de Aparato Digestivo, Hospital Comarcal de Laredo, Laredo, Cantabria, España.
Gastroenterol Hepatol. 2015 Jun-Jul;38(6):398-408. doi: 10.1016/j.gastrohep.2015.02.007. Epub 2015 Apr 1.
Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome.
肝肺综合征的特征是存在肝脏疾病、肺血管扩张和动脉血氧不足。它通常与任何病因的肝硬化相关,但在其他急慢性肝脏疾病中也有描述,且并非总是与门静脉高压相关。检测肺血管扩张的金标准方法是盐水增强超声心动图,这对肝肺综合征的诊断至关重要。这些扩张反映了肺微血管的变化(血管扩张、血管内单核细胞积聚和血管生成),并导致通气/灌注不匹配,甚至真正的肺内分流,最终引发低氧血症。该综合征会恶化患者的预后,损害其生活质量,并可能导致需要进行肝移植,而肝移植是唯一有效且确定的治疗方法。在本文中,我们综述了该综合征的病因、病理生理、临床和治疗特征。