Liver Disease in Patients After the Fontan Operation.

Abstract

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.

DOI: 10.1016/j.amjcard.2015.11.014
05010020162017
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@article{Pundi2016LiverDI, title={Liver Disease in Patients After the Fontan Operation.}, author={Krishna N. Pundi and Kavitha N Pundi and Patrick S. Kamath and Frank Cetta and Zhuo Li and Joseph T. Poterucha and David J . Driscoll and Jonathan N Johnson}, journal={The American journal of cardiology}, year={2016}, volume={117 3}, pages={456-60} }