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Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors
  1. Jae Suk Baek1,
  2. Eun Jung Bae1,
  3. Jae Sung Ko1,
  4. Gi Beom Kim1,
  5. Bo Sang Kwon1,
  6. Sang Yun Lee1,
  7. Chung Il Noh1,
  8. Eun-Ah Park2,
  9. Whal Lee2
  1. 1Department of Pediatrics, Seoul National University Children's Hospital, Seoul, South Korea
  2. 2Department of Radiology, Seoul National University Hospital, Seoul, South Korea
  1. Correspondence to Eun Jung Bae, Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehag-Ro, Jongno-gu, Seoul 110-744, South Korea; eunjbaek{at}snu.ac.kr

Abstract

Objective To identify the prevalence, clinical characteristics, risk factors of hepatic complications after a Fontan operation.

Methods This was a cross-sectional study of 139 Fontan patients who underwent cardiac CT scans out of a total of 204 patients who had undergone the Fontan procedure between 1986 and 2003. Mean age was 19.0±6.3 years and mean elapsed time since the initial Fontan operation was 11.5±4.7 years. Subjects' clinical features, echocardiograms, radiological features and biochemical test results were reviewed. Various non-invasive hepatic fibrosis blood markers were also evaluated.

Results Fifty-seven patients had hepatic complications, including radiological features of liver cirrhosis (25.9%), thrombocytopenia (7.2%), hyperbilirubinaemia (20.9%) and hepatic masses (2.9%). Hepatic complications were also significantly associated with ventricular dysfunction (p=0.020), absence of fenestration (p=0.004), thrombus in the Fontan tract (p=0.027), sinus node dysfunction (p=0.034) and tachyarrhythmia (p<0.001). In a multivariate analysis, the elapsed time since the initial Fontan operation was the only measure that was correlated with hepatic complications; the odds ratio of the post-Fontan duration of 16–20 years to that of 0–5 years was 9.00 (CI 2.24 to 36.17). The non-invasive hepatic fibrosis blood marker (Forns index) was also correlated with the elapsed time since the initial Fontan operation (r=0.718, p<0.001).

Conclusion Late hepatic dysfunction and cirrhotic change were often seen in Fontan patients. Moreover, hepatic complications were correlated with the duration of Fontan circulation. Therefore, after a Fontan operation, regular evaluation of the hepatic condition is required—for which some non-invasive hepatic fibrosis markers can be effectively used.

  • Liver cirrhosis
  • Fontan operation
  • congenital heart defect
  • paediatric cardiology

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Footnotes

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the Seoul National University Hospital Institutional Review Board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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