A case of hereditary hemorrhagic telangiectasia complaining of shunt encephalopathy diagnosed by fast Fourier transform and contrast-enhanced ultrasonography

Abstract

A 76-year-old Japanese man with probable hereditary hemorrhagic telangiectasia (HHT) presented with hepatic arteriovenous malformation (AVM) and hyperintense T1 signal of the bilateral basal ganglia on magnetic resonance imaging. He was diagnosed with shunt encephalopathy because of depressed consciousness and drooling. Fast Fourier transform analysis and contrast-enhanced sonography showed a marked increase in blood flow with decreased arterial resistance in the hepatic artery and a delay in the transition to the Kupffer phase. This is the first report of an assessment of hepatic shunt blood flow in an HHT case with shunt encephalopathy caused by hepatic AVM.