双向上腔静脉肺动脉吻合术后并发肺动静脉异常的发病机制

The Etiopathology on Pulmonary Arteriovenous Malformations Complicated with Bidirectional Superior Cavopulmonary Anastomosis

综述肺动静脉异常发生的病理机制。双向上腔静脉肺动脉吻合术后并发肺动静脉异常是患者术后紫绀进行性加重的主要原因之一 ,其发病机制一般认为与肺循环血流动力学、肝因子或肝静脉缺乏和低氧等因素有关。双向上腔静脉肺动脉吻合术后低于正常值的动脉血氧饱和度可刺激一些血管生成因子 ,如血管内皮生长因子和成纤维细胞生长因子的表达增加 ;肝静脉血被排除在肺循环以外 ,其内在的抑血管形成因子缺乏使血管重塑 ,最终导致肺动静脉异常的形成。

To review the research progress of etiopathology on pulmonary arteriovenous malformations (PAVMs) complicated with bidirectional superior cavopulmonary anastomosis( BSCA). PAVMs complicated with BSCA is one of the main reason of progressive cyanosis after operation. The etiopathology on PAVMs was general regarded with the relation of pulmonary circulation hemodynamics, absence of hepatic factor or hepatic vein and hypoxia. The summary of the above may be that oxygen saturation of artery blood less than normal stimulates the increase of some angiogenesis factor expression,such as vascular endothelial growth factor and fibroblast growth factor; the absence of inhibiting angiogenesis factor in the hepatic vein blood, when it is excluded from pulmonary circulation leads to vascular remodeling; and all these make PAVMs ultimately.