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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12890-019-0788-8) contains supplementary material, which is available to authorized users.