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Koteda Y et al. - An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. To control blood ammonia level, the authors placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device.

Exclusive Author Commentary
Kenji Suda, 05/27/09

This report indicates that there may be more patients with heterotaxy syndrome with latent portal-systemic shunt than people thought. In addition, these patients may suffer from portal-systemic encephalopathy late after Fontan-type operation because many patients with a Fontan-type operation showed early signs of liver fibrosis or real cirrhosis in later life.

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