PREVALENCE AND PATTERN OF HEPATIC DYSFUNCTION FOLLOWING FONTAN PROCEDURE

Fontan手术提供了一种缓解与一些复杂的先天性心脏病病变相关的血流动力学紊乱的方法。它是通过利用全身静脉压驱动血液通过肺毛细血管床来实现的。结果，在大多数情况下，是一个长期升高的全身静脉压。尽管有几位作者对这种全身性静脉高压对肝脏的长期影响提出了担忧，但没有人对接受Fontan手术的患者的肝功能进行系统评估，也没有人对这些患者的生化和生理异常进行精确的描述。我们建议在横断面研究中确定接受Fontan手术的患者的肝血流特征（半乳糖清除率、多普勒超声）和肝合成功能（PT、氨、胆固醇、凝血因子）。在本研究的第二阶段，我们将进行一项纵向研究，描述丰坦后PT和半乳糖消除随时间的变化，以确定这些测试是否可用于监测这些患者肝功能障碍的发展。

The Fontan procedure offers a means of palliating the hemodynamic derangements associated with some complex congenital heart lesions. It does so by utilizing systemic venous pressure to drive blood across the pulmonary capillary bed. The result, in most cases, is a chronically elevated systemic venous pressure. Although several authors have raised concerns about the long term effect of this systemic venous hypertension on the liver, no one has systemically evaluated hepatic function in patients who have undergone the Fontan procedure nor characterized the precise biochemical and physiologic abnormalities in these patients. We propose to determine in a cross sectional study the characteristics of hepatic blood flow (galactose clearance, doppler ultrasonography), and hepatic synthetic function (PT, ammonia, cholesterol, clotting factors) in patients who have undergone the Fontan procedure. In the second phase of this study, we will perform a longitudinal study to describe the changes in PT and galactose elimination over time post Fontan to determine if these tests may be useful in monitoring the development of hepatic dysfunction in these patients.