Bentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection

Neoaortic root dilation is a common phenomenon after the Norwood procedure, but the real incidence and its natural history are unclear. Regular surveillance in these patients after the operation is necessary. Herein, we present an 11-year-old boy born with tricuspid atresia, a discordant ventriculo-arterial connection and a hypoplastic aortic arch, who was palliated initially with a hybrid stage I procedure involving a reversed Blalock-Taussig shunt, followed by comprehensive stage II and then, an extra-cardiac fenestrated Fontan operation. The patient developed an aortic root aneurysm and severe aortic regurgitation. He was electively taken into the operating room, where cardiopulmonary bypass was established through a peripheral cannulation of the femoral vessels due to the high risk nature of the reoperation. A mechanical Bentall procedure was performed without residual lesions and the native ascending aorta was anastomosed as a single coronary button to the anterior wall of the graft.