Hypoxia due to intrapulmonary vascular dilatation in a toddler with a congenital portacaval shunt: case report.

Background: The term hepatopulmonary syndrome typically applies to cyanosis that results from "intrapulmonary vascular dilatation" due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen.

Case Presentation: Here, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. A transcatheter embolization of the shunt resulted in resolution of his findings.

Conclusions: Congenital portosystemic shunts need to be considered in the differential diagnosis of cyanosis.