Lena E. Trager

By Lena E. Trager and Sameh M. Said

Congenital aortic valve stenosis represents 3–5% of patients with congenital heart disease. Management options include both transcatheter and surgical. Open valvotomy/valvuloplasty and aortic valve replacement represent the main surgical choices, and while aortic valve repair is preferred, replacement may be the only option for non-repairable valves. Current available replacement options include pulmonary autograft, homografts, and biological or mechanical prostheses. The Ross procedure first introduced in 1967 by Donald Ross utilizes the patient’s pulmonary valve (autograft) as an aortic valve substitute. Despite being technically challenging it carries the advantages of maintaining the growth potentials and freedom from anticoagulation which are important in young patients. The procedure gained wide interest initially, however it fell out of favor due to concerns related to its complexity and risks of creating “two-valve” disease. Recently, long-term data confirmed the Ross procedure excellent outcomes and better survival in comparison to other aortic valve replacement options. As a result, currently it is considered the procedure of choice for young adults with congenital aortic valve stenosis at many institutions. This chapter discusses the technical aspects of the Ross procedure, and its modifications, and available options for the failing Ross, in addition to outcomes and future directions.

Part of the book: Aortic Stenosis