Unicuspid Aortic Valve with Bilateral Superior Vena Cavae, Abnormal Coronary Anatomy, and Right Ventricular Clot: A Rare Case Report

A teenager presented with congestive heart failure due to severe aortic stenosis. She had also developed ventricular clots due to ventricular dysfunction, therefore, was taken up for emergency aortic valve replacement. To our surprise, she had bilateral superior vena cava, which has not been reported with unicuspid aortic valve and ventricular clots, to the best of our knowledge. She also had an abnormal location of coronary ostia making them susceptible to injury during routine aortotomy, but it was picked up during transesophageal echocardiography and a careful aortic incision was planned. This report emphasizes the importance of careful aortotomy in congenital aortic pathologies, where coronary ostia may be located in abnormal positions. The role of transesophageal echocardiography is paramount in this situation to find the location of coronary ostia before incising the aorta.