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.
Baek
Jae Suk
JS
Department of Pediatrics, Seoul National University Children's Hospital, Jongno-gu, Seoul 110-744, South Korea.
Bae
Eun Jung
EJ
Ko
Jae Sung
JS
Kim
Gi Beom
GB
Kwon
Bo Sang
BS
Lee
Sang Yun
SY
Noh
Chung Il
CI
Park
Eun-Ah
EA
Lee
Whal
W
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