Advances in the Management of Congenital Malformations of the Aortic Valve

Congenital aortic valve disease is a life-long condition that can require multiple interventions. It is one of the most common causes of congenital heart defect, with bicuspid aortic valve present in at least 1−2% of the general population. Surgical management of congenital aortic valve disease consists of either valve repair or replacement. While aortic valve replacement using the Ross procedure can be considered the gold standard management in the pediatric population, advancements in aortic valve repair techniques have proved its usefulness as an initial management approach as it prevents prosthesis-related complications and patient-prostheses mismatch while the patient grows. Overall, all techniques have their benefits and limitations in terms of growth potential, durability of repair, freedom from reoperation and anticoagulation, infection risk, and mortality. Each patient will require an individualized judiciously selected management plan to minimize the number of interventions over their lifetime. The aim of this review is to discuss the merits and drawbacks of the major techniques currently used in both aortic valve repair and replacement