Late hepatic complications after Fontan operation; non-invasive markers of hepatic fibrosis and risk factors.
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).


CONCLUSIONS
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.
Department of Pediatrics, Seoul National University Children's Hospital, Jongno-gu, Seoul 110-744, South Korea.
Jae Suk
JS
Baek
Eun Jung
EJ
Bae
Jae Sung
JS
Ko
Gi Beom
GB
Kim
Bo Sang
BS
Kwon
Sang Yun
SY
Lee
Chung Il
CI
Noh
Eun-Ah
EA
Park
Whal
W
Lee
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D007432:Intraoperative Period; D008103:Liver Cirrhosis / Q000000981:diagnostic imaging / Q000209:etiology; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D012307:Risk Factors; D013921:Thrombocytopenia / Q000209:etiology; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D015415:Biomarkers