Friedrich-Rust Mireen, Koch Constanze, Rentzsch Axel, Sarrazin Christoph, Schwarz Peter, Herrmann Eva, Lindinger Angelika, Sarrazin Ulrike, Poynard Thierry, Schäfers Hans-Joachim, Zeuzem Stefan, Abdul-Khaliq Hashim
Department of Internal Medicine, JW Goethe University Clinic, Frankfurt, Germany.
J Thorac Cardiovasc Surg. 2008 Mar;135(3):560-7. doi: 10.1016/j.jtcvs.2007.09.039.
Studies have shown that patients with failing Fontan circulation may develop liver cirrhosis with its sequelae. Therefore, early detection of fibrosis is essential to identify patients at risk. Transient elastography has been evaluated for noninvasive staging of liver fibrosis in a multitude of studies.
A total of 39 patients who underwent the Fontan procedure were included in the study. All patients underwent an abdominal ultrasound, transient elastography, and detailed laboratory testing. Conventional echocardiography assessment of inflow and outflow and ventricular function were performed.
Significant signs of liver fibrosis were found in 36 of 39 children according to the elastography method and in 28 of 39 children according to the measured biochemical fibrosis markers. The Spearman correlation coefficient between the liver stiffness measured with transient elastography and the time interval since the Fontan operation was highly significant (0.514, P = 0.001).
The present study shows that patients who undergo the Fontan procedure are at increased risk of developing liver fibrosis and liver cirrhosis. The risk increases with the age of the patient and the time interval since the Fontan procedure. The noninvasive measurement of liver fibrosis using transient elastography and fibrosis marker scores can be a useful tool to identify patients at risk and for noninvasive surveillance.
研究表明，存在功能性单心室循环衰竭的患者可能会发展为肝硬化及其后遗症。因此，早期检测纤维化对于识别有风险的患者至关重要。在众多研究中，已对瞬时弹性成像用于肝纤维化的无创分期进行了评估。
本研究共纳入39例行Fontan手术的患者。所有患者均接受了腹部超声、瞬时弹性成像及详细的实验室检查。进行了常规超声心动图对流入、流出及心室功能的评估。
根据弹性成像方法，39名儿童中有36名发现了明显的肝纤维化迹象；根据所测生化纤维化标志物，39名儿童中有28名发现了明显的肝纤维化迹象。瞬时弹性成像测得的肝脏硬度与Fontan手术后的时间间隔之间的Spearman相关系数非常显著（0.514，P = 0.001）。
本研究表明，行Fontan手术的患者发生肝纤维化和肝硬化的风险增加。风险随患者年龄及Fontan手术后的时间间隔增加。使用瞬时弹性成像进行肝纤维化的无创测量及纤维化标志物评分可成为识别有风险患者及进行无创监测的有用工具。
