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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.

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