Department of Gastroenterology, Hepatology and Liver Transplant, Queensland Children's Hospital, Brisbane, Australia.
Department of Cardiology, Queensland Children's Hospital, Brisbane, Australia.
J Pediatr Gastroenterol Nutr. 2024 Jul;79(1):126-130. doi: 10.1002/jpn3.12182. Epub 2024 Mar 13.
The spectrum of Fontan-associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear-wave elastography (SWE) in predicting the presence of advanced FALD. Forty-eight patients (30 males, 13.9 [6-21] years) with a Fontan circulation were evaluated at 8.3 (2.1-18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5-38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post-Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase-to-platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD.
法洛四联症相关肝病（FALD）的范围从肝功能异常到纤维化甚至肝硬化不等。在这项前瞻性研究中，我们评估了剪切波弹性成像（SWE）在预测晚期 FALD 中的作用。48 名（30 名男性，13.9[6-21]岁）Fontan 循环患者在 Fontan 手术后 8.3（2.1-18.7）年接受评估。中位肝脏硬度测量值（LSM）值高于正常儿童，为 15.4（9.5-38.7）kPa。LSM 值与测量时的年龄（r=0.25，p=0.01）和 Fontan 手术后的时间（r=0.31，p=0.02）有弱但显著的相关性。它与同时进行的天门冬氨酸转氨酶/血小板比值指数相关性较差（r=0.1，p=0.39）。在有或没有超声晚期肝病证据的儿童之间，弹性成像值没有差异（17.7[四分位间距，IQR：4]vs.16.1[IQR：6]，p=0.62）。需要进一步的研究来确定 SWE 作为 FALD 肝纤维化的非侵入性标志物的确切作用。
