Over the past 3 decades, aortic root replacement surgery has evolved with much attention given to preserving the aortic valve when possible. Valve-sparing operations including the reimplantation and remodeling procedures, along with modifications, have gained support with the techniques being expanded to treat aneurysmal aortic disease in children. The benefits of conserving the valve are clear: avoidance of complications associated with anticoagulation therapy. Concerns over the development of aortic valve insufficiency or a pseudoaneurysm necessitating further surgery have been the primary arguments against these operations.
Midaortic syndrome (MAS) is narrowing of the descending thoracoabdominal aorta. Symptomatic MAS is associated with high mortality in neonates and infants.1 We describe the case of a 4-month-old infant with MAS and illustrate a staged, multidisciplinary, and multimodality approach.