Novel Staged Device Occlusion of Portosystemic Shunt With Atrial Flow Regulator in IVC Stent Platform.

There may be significant hypoplasia of the intrahepatic portal venous system. 1 Clinical manifestations include hepatic encephalopathy, liver nodules, hepatopulmonary syndrome, and portopulmonary hypertension. 2 Portopulmonary hypertension is present in 13% to 66% with varying degrees of severity, with the risk likely increasing with age and shunt size. 3 , 4 The pathophysiology is multifactorial.

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