Portal hypertension secondary to intrahepatic arterio-portal shunt in primary amyloidosis: a case report.

Portal hypertension is a rare complication in hepatic amyloidosis. We experienced a case of a 40-year-old man with primary amyloidosis and advanced esophageal varices. Angiographic procedures clearly demonstrated portal hypertension secondary to intrahepatic arterio-portal shunting. Hepatic arterial embolization brought about a disappearance of the A-P shunt and portal hypertension, though rebleeding occurred. To our knowledge, this is the first case report in which the pathogenesis of portal hypertension in hepatic amyloidosis was elucidated.