Hepatic Adenomatosis in a 7-Year-Old Child Treated Earlier With a Fontan Procedure

• Published in: Pediatric cardiology 2010-08, Vol.31 (6), p.861-864
• Main Authors: Babaoglu, Kadir, Binnetoglu, Fatih Köksal, Aydoğan, Ayşen, Altun, Gürkan, Gürbüz, Yeşim, İnan, Nagihan, Çorapçıoğlu, Funda
• Format: Article
• Language: English
• Subjects: Adenoma, Liver Cell - complications; Adenoma, Liver Cell - diagnosis; Biopsy; Cardiac Surgery; Cardiology; Case Report; Child; Diagnosis, Differential; Echo-Planar Imaging; Female; Fontan Procedure - methods; Heart Defects, Congenital - complications; Heart Defects, Congenital - surgery; Humans; Hypertension; Liver cirrhosis; Liver Neoplasms - complications; Liver Neoplasms - diagnosis; Medicine; Medicine & Public Health; Time Factors; Vascular Surgery
• ISSN: 0172-0643; 1432-1971
• DOI: 10.1007/s00246-010-9685-x

Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors’ knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure.