A catheter-based interventional strategy redirects hepatic vein flows after Fontan procedure in left isomerism to treat severe hypoxemia

Abstract

Pulmonary arteriovenous malformations after the Kawashima procedure causing severe hypoxemia are treated by Fontan surgery that redirects hepatic venous blood to the pulmonary circulation. Alignment of the hepatic venous conduit toward both pulmonary arteries is crucial for their regression. Persistent hypoxemia due to nonregressed malformations in one lung often warrants a repeat surgery to redirect hepatic venous flows. Catheter-based redirection of Fontan flows is not commonly performed as metallic stents and devices may predispose to thrombus formation in the circuit. This report highlights a patient with persistent arteriovenous malformations in the left lung leading to severe disabling hypoxemia that was managed in the catheterization laboratory with an innovative strategy to redirect Fontan flows toward the affected left lung.

Keywords

• azygos continuation
• extracardiac conduit fontan surgery
• heterotaxy
• hypoxemia
• left isomerism
• redirection of fontan flows