Abadeer Maher, Greer Joshua, Reddy Surendranath, Divekar Abhay, Schooler Gary R, Fares Munes, Dillenbeck Jeanne, Philip Steven, Zabala Luis, Sharma Bharti, Hussain Tarique
Department of Pediatric Cardiology, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
Department of Pediatric Cardiology, UT Southwestern Medical Center, Dallas, TX, USA.
Pediatr Cardiol. 2025 Apr 15. doi: 10.1007/s00246-025-03854-0.
Predictors of Fontan-associated liver disease (FALD) are not well defined. We hypothesized that magnetic resonance elastography liver stiffness measurement (LSM) would correlate with cardiac MRI findings, and hemodynamic, clinical, radiographic, and laboratory measures of FALD and Fontan failure. Patients undergoing pre- and post- Fontan assessment with same-day MRI and cardiac catheterization were enrolled. Data was collected retrospectively. 22 Fontan (mean age 12.1 ± 7.5 years) and 17 Glenn patients (mean age 4.0 ± 0.9 years) were retrospectively enrolled. LSM was higher in Fontan vs Glenn patients (3.8 ± 0.6 kPa vs 2.2 ± 0.4 kPa; p < 0.0001), as were all fibrosis scores (FIB- 4 = 0.4 0.2 vs 0.1 0.1, respectively, p = 0.002). LSM positively correlated with hepatic wedge pressure (r = 0.75; p < 0.0001), and all fibrosis scores (FIB- 4; r = 0.55; p = 0.001). There was negative correlation with LSM and ejection fraction (r = - 0.64; p < 0.0001). Comparing those with Fontan failure (n = 8) and those without (n = 14), only ejection fraction was statically significant (41 ± 12% vs 51 ± 4%, respectively; p = 0.012). Of the patients in Fontan failure, there was no difference in LSM between those with FALD (n = 3), and those without (n = 5), (3.7 0.3 kPa vs 3.8 0.6 kPa; p = 0.72). On multivariable regression analysis, LSM and GGT were independently associated with elevated hepatic venous pressure (p = 0.004 and p = 0.016, respectively). There was no correlation with LSM and Fontan duration (r = 0.15; p = 0.517). Liver stiffness rises significantly after Fontan palliation and positively correlates with hemodynamic and clinical markers of hepatic congestion and fibrosis, however its ability to predict Fontan failure and FALD remains uncertain.
Fontan相关肝病（FALD）的预测因素尚未明确界定。我们推测磁共振弹性成像肝脏硬度测量（LSM）将与心脏磁共振成像结果、以及FALD和Fontan衰竭的血流动力学、临床、影像学和实验室指标相关。纳入同日接受MRI和心导管检查的Fontan手术前后评估的患者。数据进行回顾性收集。回顾性纳入了22例Fontan患者（平均年龄12.1±7.5岁）和17例Glenn手术患者（平均年龄4.0±0.9岁）。Fontan患者的LSM高于Glenn患者（3.8±0.6kPa对2.2±0.4kPa；p<0.0001），所有纤维化评分也是如此（FIB-4分别为0.4±0.2对0.1±0.1，p=0.002）。LSM与肝楔压呈正相关（r=0.75；p<0.0001），与所有纤维化评分（FIB-4；r=0.55；p=0.001）也呈正相关。LSM与射血分数呈负相关（r=-0.64；p<0.0001）。比较Fontan衰竭患者（n=8）和无Fontan衰竭患者（n=14），只有射血分数有统计学意义（分别为41±12%对51±4%；p=0.012）。在Fontan衰竭患者中，有FALD患者（n=3）和无FALD患者（n=5）的LSM无差异（3.7±0.3kPa对3.8±0.6kPa；p=0.72）。多变量回归分析显示，LSM和谷氨酰转肽酶（GGT）与肝静脉压升高独立相关（分别为p=0.004和p=0.016）。LSM与Fontan手术持续时间无相关性（r=0.15；p=0.517）。Fontan姑息治疗后肝脏硬度显著升高，并与肝脏充血和纤维化的血流动力学及临床指标呈正相关，然而其预测Fontan衰竭和FALD的能力仍不确定。
