Liver Disease in Patients After the Fontan Operation
Liver Disease in Patients After the Fontan Operation
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DOI：
10.1016/j.amjcard.2015.11.014
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发表时间：
2016-02-01
影响因子：
2.8
通讯作者：
Johnson, Jonathan N.
中科院分区：
文献类型：
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作者：
Pundi, Krishna;Pundi, Kavitha N.;Johnson, Jonathan N.
我们回顾了1973年至2012年在我们机构（n = 1，138）进行首次Fontan手术或翻修的所有患者的记录; 195例患者有术后肝脏数据可用。肝硬化是通过组织病理学或影像学特征性发现确定的，并由肝病学家诊断为肝硬化。在195例活检或影像学检查的患者中，10年、20年和30年无肝硬化的比例分别为99%、94%和57%。195例患者中有40例（21%）诊断为肝硬化（Fontan时平均年龄为10.7 ± 8岁）。在多变量分析中，左心发育不良综合征与肝硬化风险增加相关（n = 2/16，p = 0.0133），而术前窦性心律具有保护作用（p = 0.009）。诊断为肝硬化后1年和5年的生存率分别为57%和35%。9例患者的死亡原因已知（5例多器官衰竭、2例肝衰竭和2例心力衰竭）。总之，Fontan术后肝硬化的发生率增加，在设计Fontan术后患者的随访方案时应考虑到这一点。(C)2016 Elsevier Inc. All rights reserved.
We reviewed records of all patients with an initial Fontan operation or revision from 1973 to 2012 at our institution (n = 1,138); 195 patients had postoperative liver data available. Cirrhosis was identified by histopathology or characteristic findings on imaging with an associated diagnosis of cirrhosis by a hepatologist. Of 195 patients with biopsy or imaging, 10-, 20-, and 30 -year freedom from cirrhosis was 99%, 94%, and 57%, respectively. There were 40 of 195 patients (21%) diagnosed with cirrhosis (mean age at Fontan 10.7 +/- 8 years). On multivariate analysis, hypoplastic left heart syndrome was associated with increased risk of cirrhosis (n = 2 of 16, p = 0.0133), whereas preoperative sinus rhythm was protective (p = 0.009). Survival after diagnosis of cirrhosis was 57% and 35%, at 1, and 5 years, respectively. The cause of death was known for 9 patients (5 multiorgan failure, 2 liver failure, and 2 heart failure). In conclusion, there is an incremental occurrence of cirrhosis after the Fontan, which should be considered when designing follow-up protocols for patients after Fontan operation. (C) 2016 Elsevier Inc. All rights reserved.