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 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.
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References
Arcidi JM Jr, Moore GW, Hutchins GM (1981) Hepatic morphology in cardiac dysfunction: a clinicopathologic study of 1,000 subjects at autopsy. Am J Pathol 104:159–166
Cromme-Dijkhuis AH, Hess J, Hählen K, Henkens CM, Bink-Boelkens MT, Eygelaar AA, Bos E (1993) Specific sequelae after Fontan operation at mid- and long-term follow-up: arrhythmia, liver dysfunction, and coagulation disorders. J Thorac Cardiovasc Surg 106:1126–1132
Flejou JF, Barge J, Menu Y, Degott C, Bismuth H, Potet F, Benhamou JP (1985) Liver adenomatosis: an entity distinct from liver adenoma? Gastroentorology 83:1132–1138
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
Ghaferi AA, Hutchins GM (2005) Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 129:1348–1352
Gokhale R, Whitington PF (1996) Hepatic adenomatosis in an adolescent. J Pediatr Gastroenterol Nutr 23:482–486
Grazioli L, Federle MP, Ichikawa T, Balzano E, Nalesnik M, Madariaga J (2000) Liver adenomatosis: clinical, histopathologic, and imaging findings in 15 patients. Radiology 216:395–402
Jenkins JG, Lynn AM, Wood AE, Trusler GA, Barker GA (1982) Acute hepatic failure following cardiac operation in children. J Thorac Cardiovasc Surg 84:865–871
Kiesewetter CH, Sheron N, Vettukattill JJ, Hacking N, Stedman B, Millward-Sadler H, et al (2007) Hepatic changes in the failing Fontan circulation. Heart 93:579–584
Lemmer JH, Coran AG, Behrendt DM, Hiedelberger KP, Stern AM (1983) Liver fibrosis (cardiac cirrhosis) five years after modified Fontan operation for tricuspid atresia. J Thorac Cardiovasc Surg 86:757–760
Matsuda H, Covino E, Hirose H, Nakano S, Kishimoto H, Miyamoto Y, Nishigaki K, Takano H, Ohtake S, Sakaki S et al (1988) Acute liver dysfunction after modified Fontan operation for complex cardiac lesions: analysis of the contributing factors and its relation to the early prognosis (abstract). J Thorac Cardiovasc Surg 96:219–226
Narkewicz MR, Sondheimer HM, Ziegler JW, Otanni Y, Lorts A, Shaffer EM, Horgan JG, Sokol RJ (2003) Hepatic dysfunction following the Fontan procedure. J Pediatr Gastroenterol Nutr 36:352–357
Saliba T, Dorkhom S, O’reilly EM, Ludwig E, Gansukh B, Abou-Alfa GK (2009) Hepatocellular carcinoma in two patients with cardiac cirrhosis. Eur J Gastroenterol Hepatol June 22 [Epub ahead of print]
Skarupa DJ, Ellison EC, Vitellas KM, Frankel WL (2004) Hepatocellular adenomatosis is a rare entity that may mimic other hepatocellular lesions. Ann Diagn Pathol 8:43–49
Wernovsky G, Stiles KM, Gauvreau K, Gentles TL, DuPlessis AJ, Bellinger DC, Walsh AZ, Burnett J, Jonas RA, Mayer JE Jr, Newburger JW (2000) Cognitive development after the Fontan operation. Circulation 102:883–889
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Babaoglu, K., Binnetoglu, F.K., Aydoğan, A. et al. Hepatic Adenomatosis in a 7-Year-Old Child Treated Earlier With a Fontan Procedure. Pediatr Cardiol 31, 861–864 (2010). https://doi.org/10.1007/s00246-010-9685-x
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DOI: https://doi.org/10.1007/s00246-010-9685-x