Liver Disease in Patients After the Fontan Operation

被引:132
作者
Pundi, Krishna [1 ]
Pundi, Kavitha N. [2 ]
Kamath, Patrick S. [3 ]
Cetta, Frank [2 ,4 ]
Li, Zhuo [5 ]
Poterucha, Joseph T. [2 ]
Driscoll, David J. [2 ]
Johnson, Jonathan N. [2 ,4 ]
机构
[1] Mayo Clin, Coll Med, Rochester, MN USA
[2] Mayo Clin, Dept Pediat, Div Pediat Cardiol, Rochester, MN USA
[3] Mayo Clin, Dept Med, Div Gastroenterol & Hepatol, Rochester, MN USA
[4] Mayo Clin, Dept Med, Div Cardiovasc Dis, Rochester, MN USA
[5] Mayo Clin, Div Biomed Stat & Informat, Rochester, MN USA
关键词
LONG-TERM SURVIVAL; CARDIAC CIRRHOSIS; TRICUSPID ATRESIA; HEART-TRANSPLANT; MORTALITY; PREDICTORS; PATHOLOGY; FIBROSIS;
D O I
10.1016/j.amjcard.2015.11.014
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:456 / 460
页数:5
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