Hepatic encephalopathy due to aorto-right ventricular fistula responsive to percutaneous repair: a case report

Encephalopathy due to hyperammonemia is most often found in a setting of cirrhosis. However, it can also result from increased hepatic venous pressures, which can damage zone three hepatocytes and result in elevated serum ammonia.

This report focuses on the unique case of a 43-year-old woman, who presented with confusion in the setting of hyperammonemia due to congestive hepatopathy from an iatrogenic aorto-right ventricular fistula. The patient underwent percutaneous repair of the fistula with resolution of encephalopathy and notable improvement in symptoms. The patient attended all follow-up appointments and was contacted five and eight months after admittance for updates regarding her recovery and permission to publish this case.

This exceedingly rare case has not been reported in the literature and highlights the historically narrow differential for hyperammonemic encephalopathy given the prevalence of cirrhosis and potential reversibility of such a case.