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J Thorac Cardiovasc Surg 2005;129:1348-1352
© 2005 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease |
Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Md.
Received for publication August 2, 2004; revisions received September 25, 2004; accepted for publication October 11, 2004. * Address for reprints: Grover M. Hutchins, MD, Department of Pathology, The Johns Hopkins Hospital, Pathology B-100, 600 N Wolfe St, Baltimore, MD 21287-6901. (Email: ghutchi{at}jhmi.edu).
BACKGROUND: Staged palliative surgical procedures have been an effective treatment of complex congenital heart defects. The Fontan procedure has been of particular benefit to infants with functional single-ventricle complexes but with the consequence of a sustained increase of right-sided venous pressure.
METHODS: We reviewed the clinical and pathologic features of 9 autopsied patients having undergone the Fontan procedure, with special attention given to their liver pathology.
RESULTS: The 9 patients died from a few hours to 18 years after the Fontan operation. Chronic passive congestion was seen in 7 patients, and 4 patients surviving 4 to 18 years also had cardiac cirrhosis. Hepatic adenoma in the setting of cardiac cirrhosis was found in a patient surviving for 9 years. One patient surviving for 18 years had hepatocellular carcinoma superimposed on cardiac cirrhosis. Rupture of the hepatoma in this case led to fatal hemorrhage.
CONCLUSION: The study shows that chronically increased hepatic venous pressure from the Fontan procedure might lead to chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma.
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