Abstract
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.
Highlights
Fontan procedure is a palliative surgery done as a final stage treatment in patients with single ventricle physiology [1]
Reports of intrahepatic venovenous collateral (VVC) presenting with profound cyanosis following the Fontan operation are rare and usually associated with visceral heterotaxy and a nest of intrahepatic venous plexus
We report a rare case of a fistula between a right hepatic vein and a rightsided pulmonary venous atrium in a child with visceral heterotaxy and profound hypoxemia following the Fontan operation
Summary
Fontan procedure is a palliative surgery done as a final stage treatment in patients with single ventricle physiology [1]. Other causes of hypoxemia following the Fontan operation include a residual atrial septum defect or fenestration, intrapulmonary arteriovenous fistulae, and a right to left shunt due to abnormal systemic venous drainage to the heart or the pulmonary veins. We report a rare case of a fistula between a right hepatic vein and a rightsided pulmonary venous atrium in a child with visceral heterotaxy and profound hypoxemia following the Fontan operation. Cardiac catheterization post-Fontan revealed a fistula between a right hepatic vein and a right-sided pulmonary venous atrium. Occlusion of the fistula with a 12 mm TYSHAK® balloon dilatation catheter resulted in a trivial increase in the mean pulmonary pressure from 14 mm Hg to 16 mm Hg with a rise in the arterial oxygen saturation to the high 90s with appropriate systemic blood pressure. Three years following fistula's occlusion, the patient was doing well and reporting good exercise tolerance
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