CASE REPORT Hepatic Adenomatosis in a 7-Year-Old Child Treated Earlier With a Fontan Procedure Kadir Babaoglu • Fatih Ko ¨ksal Binnetoglu • Ays ¸en Aydog ˘an • Gu ¨ rkan Altun • Yes ¸im Gu ¨rbu ¨z • Nagihan _ Inan • Funda C ¸ orapc ¸ ıog ˘lu Received: 30 December 2009 / Accepted: 10 February 2010 / Published online: 4 March 2010 Ó Springer Science+Business Media, LLC 2010 Abstract 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 hepa- topathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathol- ogies 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 reso- nance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors’ knowledge, this case involved the youngest patient with hepatocellular adenomatosis docu- mented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure. Keywords Cardiac cirrhosis Á Fontan procedure Á Hepatic adenomatosis The Fontan procedure is a palliative procedure first per- formed in 1968 [4]. In the follow-up evaluation of patients after they have undergone total cava-pulmonary connection, the average central venous pressure is found to be about three to four times higher than normal [10, 12]. Central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction are observed after this procedure. These hemodynamic changes combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. In addition, myocardial dys- function, diminished exercise capacity, arrhythmias, pro- tein-losing enteropathy, thrombotic complications, somatic growth retardation and neurodevelopmental delay are other important outcomes of this procedure [15]. The histopathologic changes of cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis [1, 5, 9, 12]. Hepatic adenoma and hepatic adenomatosis (HA), pathologies also observed dur- ing the course of cardiac hepatopathy, are not well-known. We report the case of a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Case Report A 7-year-old girl at birth had a diagnosis of congenital heart disease involving pulmonary atresia with intact ventricular K. Babaoglu (&) Á F. K. Binnetoglu Á G. Altun Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey e-mail: babaogluk@yahoo.com A. Aydog ˘an Department of Pediatric Gastroenterology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey Y. Gu ¨rbu ¨z Department of Pathology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey N. _ Inan Department of Radiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey F. C ¸ orapc ¸ ıog ˘lu Department of Pediatric Oncology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey 123 Pediatr Cardiol (2010) 31:861–864 DOI 10.1007/s00246-010-9685-x