Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes

Many children-particularly those with Williams syndrome-show regression of stenosis without intervention. Children who undergo operation have high LVOT gradients and smaller LVOT z scores that do not improve over time. Surgical intervention alters the natural history: LVOT obstruction is relieved and does not recur, and ascending aortic dimensions progressively enlarge towards normal values.