Bicuspid aortic valve (BAV) disease is the most common form of congenital heart disease, affecting 1-2% of the population. Only 20% of patients will maintain normal valve function throughout their life and more than 30% of patients will develop serious morbidity. It is a highly heritable condition, with transmission likely to be autosomal dominant. Patients with BAV have a 10-fold risk of aortic dissection when compared to the normal population. Management of BAV associated aortopathy represents a significant clinical challenge.