title: 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.
authors.affiliation: Mayo Clinic College of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Krishna
authors.initials: K
authors.lastname: Pundi
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota.
authors.forename: Kavitha N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Patrick S
authors.initials: PS
authors.lastname: Kamath
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Li
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota.
authors.forename: Joseph T
authors.initials: JT
authors.lastname: Poterucha
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota.
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota; Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address: Johnson.Jonathan@mayo.edu.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Johnson
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D005544:Forecasting; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D015994:Incidence; D007223:Infant; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D014481:United States / Q000453:epidemiology; D055815:Young Adult