Hepatic changes in the failing Fontan circulation
Hepatic changes in the failing Fontan circulation
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DOI：
10.1136/hrt.2006.094516
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发表时间：
2007-05-01
期刊：
影响因子：
5.7
通讯作者：
Veldtman, Gruschen R.
中科院分区：
文献类型：
--
作者：
Kiesewetter, Christoph H.;Sheron, Nick;Veldtman, Gruschen R.
背景：Fontan循环衰竭与肝损害有关。这种肝损伤的性质是不明确defined.Objective：建立与Fontan循环相对于临床，生化和血流动力学findings.Methods：患者的大体和组织学肝脏变化进行了回顾性评估，2003年9月至2005年6月之间的心外Fontan转换，根据一个既定的临床协议。确定了12例患者，平均年龄24.6（范围15.8-43.4）岁。平均持续时间为14.1（范围6.9-26.4）years.Results：肝静脉压力较低的患者（p=0.007）和组织学检查无心源性肝硬化的患者（p=0.033）中，CT上肝区带增强（4/12）更为常见。胃食管静脉曲张（4/12）在肝静脉压力较高的患者中更常见（21（6.3）vs 12.2（2.2）mm Hg，p=0.013），并与更晚期的肝硬化相关（p=0.037）。肝硬化程度（7/12）与肝静脉压力呈正相关（r=0.83，p=0.003）。Fontan持续时间与肝纤维化程度（r=0.75，p=0.013）以及宽瘢痕（r=0.71，p=0.021）之间存在显著正相关。蛋白丢失性肠病（5/12）在Fontan持续时间较长的患者中更常见（11.7（3.2）vs 17.9（6.1）年，p=0.038）。结论：肝损伤在该患者组中可能是广泛的，与Fontan持续时间和肝静脉压力有关。CT扫描辅助非侵入性评估。心源性肝硬化伴胃食管静脉曲张和再生性肝结节（肝细胞癌的前兆）的风险在该患者组中很常见。
Background: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined.Objective: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings.Methods: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 ( range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 ( range 6.9-26.4) years.Results: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p=0.007), and in those with absent cardiac cirrhosis on histological examination (p=0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure ( 21 (6.3) vs 12.2 (2.2) mm Hg, p=0.013) and associated with more advanced cirrhosis (p=0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r=0.83, p=0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r=0.75, p=0.013), as well as presence of broad scars (r=0.71, p=0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p=0.038).Conclusions: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.