The Benefits of an Intracardiac Conduit in the Fontan Operation for Complex Systemic Venous Anomalies in Patients with Single Ventricle Physiology

Abstract

Background: Systemic venous anomalies pose challenges to cavopulmonary connections in univentricular patients. At our unit, the intracardiac conduit (ICC) Fontan has been introduced for cases involving apicocaval juxtaposition (ACJ). This addresses issues of a long-curved extracardiac conduit (ECC) to the contralateral pulmonary artery (PA) and potential compression of the ECC to the ipsilateral PA. Methods: We present four patients at various stages who underwent an ICC Fontan operation. This includes primary Glenn connections with the setup for the cath lab Fontan. Results: The ICC placement was done in four levocardic patients. The first patient had an interrupted inferior vena cava (IVC) who previously underwent the Kawashima operation on the left side with pulmonary arteriovenous malformation. The ipsilateral drainage hepatic veins (HVs) were connected to the right PA using an ICC. The second and third patients with left ACJ and preexisting bilateral Glenns underwent IVC to the right PA connection using an ICC. The fourth patient had bilateral superior vena cava, left ACJ, ipsilateral HV drainage, and presented at 10 years of age having had no prior operations. He underwent construction of a bilateral bidirectionalGlenn, right HV to conduit baffling, and IVC to the main PA connection using an ICC with two fenestrations (to allow a gradual ventricular preload change; discharge saturation of 80%). This set him up for interventional closure of the larger fenestration later for physiologic Fontan completion. All the patients recovered with unobstructed conduits and were anticoagulated without thromboembolic/bleeding events. Conclusions: The ICC Fontan operation provides a safe and effective cavopulmonary connection in selected patients with complex systemic venous anomalies.