Congenital Valvar and Supravalvar Aortic Stenosis

Abstract

Congenital left ventricular outflow tract obstruction accounts for 10% of all congenital heart disease. This heterogeneous group includes valvar aortic stenosis, the most common type, to the rare supravalvar aortic stenosis. Presentation, diagnostic work up and management strategies are dependent on age, anatomy, and physiology. In neonates with critical aortic stenosis suitable for biventricular repair both balloon and open interventions are established as an effective treatments. Management strategy in older children with valvar and/or supravalvar aortic stenosis should take into consideration factors such as growth, an active life style, and the difficulties in medical compliance. In general, the prosthetic materials should not compromise cardiac growth and life style. In particular, reconstruction of the aortic valve and pulmonary autograft procedure for replacement of the aortic valve and aortic root have dramatically changed the approach to children with congenital aortic valve disease. Alternatively, in some children the best option is still to use a mechanical prosthetic valve or allograft, despite the well-known drawbacks of these procedures. In nearly all cases, congenital left ventricular outflow tract obstruction is a life-long condition, with palliative intervention, and an ongoing need for long term follow-up.