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

• Published in: Heart (British Cardiac Society) Vol. 96; no. 21; pp. 1750 - 1755
• Main Authors: Baek, Jae Suk, Bae, Eun Jung, Ko, Jae Sung, Kim, Gi Beom, Kwon, Bo Sang, Lee, Sang Yun, Noh, Chung Il, Park, Eun-Ah, Lee, Whal
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Cardiovascular Society 01.11.2010; BMJ Publishing Group; BMJ Publishing Group LTD
• Subjects: Adolescent; Adult; Age; Biological and medical sciences; Biomarkers - blood; Blood platelets; Cardiology. Vascular system; Child; Child, Preschool; congenital heart defect; Cross-Sectional Studies; Female; Fontan operation; Fontan Procedure - adverse effects; Gallbladder diseases; Gastroenterology. Liver. Pancreas. Abdomen; Hepatitis; Histology; Humans; Hyperbilirubinemia - etiology; Hypertension; Intraoperative Period; Laboratories; Liver cirrhosis; Liver Cirrhosis - diagnostic imaging; Liver Cirrhosis - etiology; Liver Diseases - diagnostic imaging; Liver Diseases - etiology; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical imaging; Medical sciences; Morphology; Other diseases. Semiology; paediatric cardiology; Pulmonary arteries; Risk Factors; Statistical analysis; Studies; Thrombocytopenia - etiology; Thrombosis; Tomography, X-Ray Computed; Young Adult; Young adults; Hepatic fibrosis; Late; Liver; Risk factor; Non invasive method; Biological marker; Digestive diseases; Hepatic disease; Complication; Circulatory system; Cardiology; Fontan operation
• ISSN: 1355-6037; 1468-201X; 1468-201X
• DOI: 10.1136/hrt.2010.201772

ObjectiveTo identify the prevalence, clinical characteristics, risk factors of hepatic complications after a Fontan operation.MethodsThis 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.ResultsFifty-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).ConclusionLate 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.