Bicuspid aortic valve with severe stenosis and ruptured sinus of Valsalva aneurysm to left ventricle

Bicuspid aortic valve (BAV) is a common congenital cardiac anomaly, and rarely, it is associated with sinus of Valsalva aneurysms (SOVAs). And very infrequently, these SOVAs rupture into left side of heart. We hereby report a case of 12-year-old male with BAV with severe aortic stenosis with a large SOVA that ruptured into the left side of the heart. The anatomy was delineated with multimodality imaging; initially with two-dimensional trans-thoracic echocardiography (TTE), and later on with three-dimensional TTE and with multi detector computed tomography. Operative interventions were planned for the patient.