标题： 一种一维（1D）计算流体动力学研究关于Fontan相关性肝病（FALD） 显示英文标题

标题： A One Dimensional (1D) Computational Fluid Dynamics Study of Fontan-Associated Liver Disease (FALD)

摘要： 法洛氏循环相关肝病（FALD）是一种由于肝脏血流动力学变化和静脉充血引起的疾病。 这种疾病在左心发育不良综合征（HLHS）患者中尤为显著。 尽管HLHS患者通常可以活到成年，但由于其单心室生理（即法洛氏循环），他们的心输出量减少。 因此，他们无法向肝脏提供足够的血液。 相比之下，具有双出口右心室（DORV）且同样具有单心室循环的患者，FALD的发生率较低。 在本研究中，我们使用患者特异性的一维计算流体动力学（1D-CFD）模型来预测HLHS患者的肝脏血流动力学，并与年龄和体型匹配的DORV对照患者进行比较。 此外，我们在HLHS患者中模拟FALD条件，以预测疾病进展各个阶段的血流动力学变化。 我们的结果表明，与DORV患者相比，HLHS患者的门静脉压力更高。 随着FALD条件的进展，这种差异会更加明显。 HLHS患者的壁剪切应力（WSS）也高于正常水平，表明血管重塑。 在FALD条件下，WSS略有下降，这与门脉高压的发展一致。 灌注分析揭示了肝组织中可能发生纤维化的区域，显示在FALD条件下，由门静脉供血的整个肝组织的压力增加，流量减少。 我们的结果为法洛氏循环中的特定血流动力学变化提供了见解，这些变化可能导致FALD。 显示英文摘要

摘要： Fontan-Associated Liver Disease (FALD) is a disorder arising from hemodynamic changes and venous congestion in the liver. This disease is prominent in patients with hypoplastic left heart syndrome (HLHS). Although HLHS patients typically survive into adulthood, they have reduced cardiac output due to their univentricular physiology (i.e., a Fontan circuit). As a result, they have insufficient blood delivery to the liver. In comparison, patients with double outlet right ventricle (DORV), also having a univentricular circuit, have lower incidence of FALD. In this study, we use a patient-specific, one-dimensional computational fluid dynamics (1D-CFD) model to predict hemodynamics in the liver of an HLHS patient and compare predictions with an age- and size-matched DORV control patient. Additionally, we simulate FALD conditions in the HLHS patient to predict hemodynamic changes across various stages of disease progression. Our results show that the HLHS patient has a higher portal venous pressure compared to the DORV patient. This difference is exacerbated as FALD conditions progress. The wall shear stress (WSS) is also higher than normal for the HLHS patient, suggesting vascular remodeling. WSS decreases slightly under FALD conditions, consistent with the development of portal hypertension. Perfusion analysis gives insight into regions of liver tissue at risk for fibrosis development, showing increasing pressures and reduced flow throughout the liver tissue fed by the portal vein under FALD conditions. Our results provide insight into the specific hemodynamic changes in Fontan circulation that can cause FALD.