Liver Disease in Patients After the Fontan Operation

Krishna Pundi, Kavitha N. Pundi, Patrick Sequeira Kamath, Frank Cetta, Zhuo Li, Joseph T. Poterucha, David J. Driscoll, Jonathan N. Johnson

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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.

Original languageEnglish (US)
Pages (from-to)456-460
Number of pages5
JournalAmerican Journal of Cardiology
Volume117
Issue number3
DOIs
StatePublished - Feb 1 2016

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ASJC Scopus subject areas

  • Medicine(all)
  • Cardiology and Cardiovascular Medicine

Cite this

Pundi, K., Pundi, K. N., Kamath, P. S., Cetta, F., Li, Z., Poterucha, J. T., Driscoll, D. J., & Johnson, J. N. (2016). Liver Disease in Patients After the Fontan Operation. American Journal of Cardiology, 117(3), 456-460. https://doi.org/10.1016/j.amjcard.2015.11.014