Greidanus Paul G, Pagano Joseph J, Escudero Carolina A, Thompson Richard, Tham Edythe B
Division of Pediatric Cardiology, Stollery Children's Hospital & Mazankowski Alberta Heart Institute, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada.
Department of Biomedical Engineering, University of Alberta, Edmonton, Alberta, Canada.
CJC Pediatr Congenit Heart Dis. 2023 Mar 15;2(3):134-142. doi: 10.1016/j.cjcpc.2023.03.004. eCollection 2023 Jun.
Fontan-associated liver disease (FALD) is characterized by hepatic congestion and progressive hepatic fibrosis in patients with the Fontan operation. This condition is generally clinically silent until late, necessitating techniques for early detection. Liver T1 mapping has been used to screen for FALD, but without consideration of regional variations in T1 values.
Liver T1 measured with a liver-specific T1 mapping sequence (PROFIT1) in Fontan patients was compared with cohorts of patients with biventricular congenital heart disease (BiV-CHD) and controls with normal cardiac function and anatomy.
Liver T1 was significantly elevated in the Fontan cohort (n = 20) compared with patients with BiV-CHD (n = 12) and controls (n = 9) (781, 678, and 675 milliseconds, respectively; < 0.001), with a consistent pattern of significantly elevated T1 values in the peripheral compared with central liver regions (ΔT1 = 54, 2, and 11 milliseconds; < 0.001). PROFIT1 also yielded simultaneous T2∗ maps and fat fraction values that were similar in all groups. Fontan liver T1 values were also significantly elevated as compared with BiV-CHD and controls as measured with the cardiac (modified Look-Locker inversion) acquisitions (728, 583, and 583 milliseconds, respectively; < 0.001) and values correlated with PROFIT1 liver T1 (R = 0.87, < 0.001).
Fontan patients have globally increased liver T1 values and consistent spatial variations, with higher values in the peripheral liver regions as compared with spatially uniform values in BiV-CHD and controls. The spatial patterns may provide insight into the progression of FALD. Liver T1 mapping studies should include uniform spatial coverage to avoid bias based on slice locations in this population.
Fontan相关肝病（FALD）的特征是接受Fontan手术的患者出现肝淤血和进行性肝纤维化。这种情况在临床上通常直到晚期才会表现出来，因此需要早期检测技术。肝脏T1映射已被用于筛查FALD，但未考虑T1值的区域差异。
将采用肝脏特异性T1映射序列（PROFIT1）测量的Fontan患者的肝脏T1与双心室先天性心脏病（BiV-CHD）患者队列以及心脏功能和解剖结构正常的对照组进行比较。
与BiV-CHD患者（n = 12）和对照组（n = 9）相比，Fontan队列（n = 20）的肝脏T1显著升高（分别为781、678和675毫秒；P < 0.001），外周肝脏区域的T1值较中心区域显著升高，呈现一致的模式（ΔT1 = 54、2和11毫秒；P < 0.001）。PROFIT1还产生了同时的T2∗图和脂肪分数值，所有组的这些值相似。与采用心脏（改良Look-Locker反转）采集测量的结果相比，Fontan肝脏T1值与BiV-CHD和对照组相比也显著升高（分别为728、583和583毫秒；P < 0.001），且这些值与PROFIT1肝脏T1相关（R = 0.87，P < 0.001）。
Fontan患者的肝脏T1值整体升高且存在一致的空间差异，与BiV-CHD和对照组空间均匀的值相比，外周肝脏区域的值更高。这种空间模式可能有助于深入了解FALD的进展。肝脏T1映射研究应包括均匀的空间覆盖，以避免该人群中基于切片位置的偏差。
