title: Impact of liver disease after the fontan operation.
abstract: Liver disease is being reported with increased frequency in survivors of the Fontan operation. The clinical impact of structural hepatic abnormalities in these patients remains largely unknown. We sought to assess if, and how, cardiologists are screening for hepatic disease in these patients to evaluate for clinical or laboratory correlates of structural hepatic disease and determine the prevalence and clinical impact of such disease. Retrospective data analysis from tertiary institutions was performed. Hepatic imaging studies and serology performed over the last decade were reviewed and clinical and laboratory correlates of structural hepatic alterations on liver imaging or biopsy were sought. Outcomes were determined. In this cohort study, 53 of 60 adult survivors (88%) underwent hepatic imaging with computed tomography, magnetic resonance imaging, or ultrasound with a median number of 2 (0 to 10) studies over the past decade. The frequency of hepatic imaging varied widely with 70% of patients undergoing serial studies. Cirrhosis with or without abnormal hepatic nodules was seen in 29 of 53 patients (55%) at 18.4 ± 5.6 years after the Fontan procedure. Adverse hepatic-related outcome occurred in 22% of the entire patient cohort and was unrelated to time from Fontan operation. In conclusion, there exists significant variability in the type and timing of testing for hepatic complications after the Fontan procedure. Structural hepatic alterations are common and can be associated with significant morbidity and mortality. Routine imaging, and serologic evaluation, is recommended in all Fontan survivors.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Mattel Children's Hospital at University of California at Los Angeles, Los Angeles, California.
authors.forename: Ian
authors.initials: I
authors.lastname: Lindsay
authors.affiliation: Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
authors.forename: Joy
authors.initials: J
authors.lastname: Johnson
authors.affiliation: Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
authors.forename: Melanie D
authors.initials: MD
authors.lastname: Everitt
authors.affiliation: Division of Cardiology, Department of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
authors.forename: James
authors.initials: J
authors.lastname: Hoffman
authors.affiliation: Division of Cardiology, Department of Pediatrics, Children's Hospital & Medical Center, University of Nebraska Medical Center, Omaha, Nebraska. Electronic address: ayetman@childrensomaha.org.
authors.forename: Anji T
authors.initials: AT
authors.lastname: Yetman
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis / Q000453:epidemiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D011183:Postoperative Complications; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014057:Tomography, X-Ray Computed; D014481:United States / Q000453:epidemiology; D055815:Young Adult