BACKGROUND Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion. METHODS Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls. RESULTS Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. CONCLUSION Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures. TRIAL REGISTRATION Retrospectively registered data.

中文翻译：

---

小儿Fontan患者肝脏中的细胞外容量增加。

背景技术具有单心室生理的患者发生肝纤维化的风险增加。双向腔肺连接（BCPC）和方丹循环的患儿的病程和患病率尚不清楚。源自心血管磁共振（CMR）和T1弛张法的细胞外体积分数（ECV）反映了肝脏中的纤维化重塑和/或充血。这项研究的目的是调查具有单心室生理学经验的小儿患者是否增加天然T1和ECV作为肝纤维化/充血的标志物。方法回顾性测量具有BCPC和Fontan循环的患儿的肝脏天然T1时间和ECV，使用心脏短轴修饰的Look-Locker反转恢复序列显示肝脏，并与儿科对照进行比较。结果Fontan患者（n = 62，11.4±4.4岁，T1 762±64 ms）与BCPC患者（n = 20，2.8±0.9年，T1 645±43 ms，p = 0.04）相比，肝天然T1时间增加。两个队列的T1均高于对照组（n = 44，13.7±2.9岁，T1 604±54 ms，两者均p <0.001）。Fontan和BCPC患者的ECV分别为41.4±4.8％和36.4±4.8％（p = 0.02）。在Fontan患者中，T1值与暴露于体外循环时间（R = 0.3，p = 0.02），收缩压和舒张末期容积（R = 0.3，p = 0.04两者）相关，并且与氧饱和度和体表面积成反比（R = -0.3，两者的p = 0.04）。Fontan术后T1或ECV与中心静脉压或年龄无明显关联。结论Fontan和BCPC患者的CMR标记升高，提示肝纤维化和/或充血，即使在很小的时候。组织变化似乎与中心静脉压无关。试用注册追溯注册数据。