Isolated subpulmonary ventricular assist device support for failing Fontan circulation in hypoplastic left heart syndrome

A 13-year-old boy born with hypoplastic left heart syndrome, who underwent all three stages of univentricular palliation and multiple interstage operations, developed failing Fontan circulation with protein-losing enteropathy and massive pleural and peritoneal effusions. In this video tutorial, surgical assessment and implanting an isolated subpulmonary ventricular assist device for support using the EXCOR Venous Cannula and a 30-cc EXCOR system are described. In addition, aspects of the surgical removal of "fenestration" devices are reported. Cardiopulmonary bypass is established in a central fashion, and the procedure is conducted under mild hypothermia and partially induced ventricular fibrillation. The superior vena cava and an 18-mm extracardiac Fontan conduit are completely divided. The fenestration devices are partially removed through the opening in the right pulmonary artery. The EXCOR venous inlet cannula is first anastomosed to a rim of the Fontan graft connected to the inferior vena cava. Then it is anastomosed to the superior vena cava by means of an interposition graft. The final anastomosis connects the outflow cannula to the superior part of the former Fontan conduit.