Insights in Pediatric Cardiology

Transcatheter Occlusion of a Hepatic Vein to Left Atrium Fistula: Should we Close Venovenous Collateral Vessels Following Fontan Operation?

Right to left shunt due to abnormal systemic venous drainage to the heart or the pulmonary veins is among the causes of hypoxemia following the Fontan operation. There are conflicting data regarding the closure of the Venovenous Collateral (VVC) vessels post-Fontan. The embolization of these VVC in older patients may be associated with decreased survival.

We describe a rare fistula draining a right-sided hepatic vein connected to a hepatic venous plexus to a right-sided pulmonary venous atrium in a child with visceral heterotaxy. The patient presented with severe hypoxemia following the Fontan operation. The fistula was successfully occluded by an Amplatzer™ Vascular Plug II to resolve the hypoxemia and improve the hemodynamic status.

Approach to venovenous collaterals in older patients with mild cyanosis after the Fontan operation may differ from that in younger children with severe hypoxemia and intrahepatic VVC. Patients with profound cyanosis due to intrahepatic to atrial connection may benefit from the occlusion of these connections. Percutaneous closure of hepatic to atrial connection with significant hypoxemia following appropriate hemodynamic assessment may be beneficial.

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