Supravalvar Aortic Stenosis

Supravalvar aortic stenosisSupravalvar aortic stenosis is a rare type of left ventricular outflow tract stenosis and is most commonly localized at the sinotubular junction. It can be diffuse and extend into the distal aorta and supraaortic vessels. It can be part of more complex multilevel outflow obstructions. The disease is associated with Williams syndromeWilliams syndrome, an elastin gene mutation in 60–70% of cases. There is also an association with multiple branch pulmonary artery stenosesBranch pulmonary arterial stenosis. Intervention is indicated if significant stenosis is present or symptomatic. Surgical risk is low and indication for surgery should consider potentially quick progression of the stenosis. Various patch techniques have been reported to address different aspects of the disease. SurvivalSurvival is good, but reintervention may be necessary.