Fenestration in Extracardiac Conduits in Children After Modified Fontan Operation by Implantation of Stent Grafts

Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surgical fenestrations in two children and absent fenestration in one. As an emergency procedure, in all patients the conduit was perforated by transcatheter intervention in order to create a connection to the anatomical right atrium. Following balloon dilatation of the perforated conduit, in all three patients covered stent grafts were placed in the newly created defect to attain a reliable communication. Patency of the fenestration was demonstrated by angiogram and any leakage was ruled out. Cardiac output improved and severe pleural effusion and ascites subsided. Right-to-left shunt could be demonstrated by echocardiography at follow-up after 7 months (median) in all three patients. Oxygen saturation remained stable between 85 and 90%. These preliminary results suggest that stent graft fenestration can serve as a valuable tool in failing Fontan circulation, particularly in patients with an extracardiac conduit. Covered stents have the potential to reduce the acute risk of bleeding and they help to prevent early spontaneous closure of the newly created fenestration.