Surgical Repair of Severe Supravalvular Aortic Stenosis with Bicuspid Aortic Valve and Single Coronary Artery in a 5-Year-Old Child

Background: Supravalvular aortic stenosis (SVAS) is a rare form of left ventricular outflow tract obstruction in children. Its coexistence with a bicuspid aortic valve (BAV) and a single coronary artery is uncommon and presents significant surgical challenges. Case Summary: We report a 5-year-10-month-old child with severe SVAS associated with BAV, post-stenotic aortic dilatation, and a single right coronary artery giving rise to the left coronary artery. Multidetector CT angiography confirmed the anomalous coronary anatomy. The patient underwent median sternotomy, supravalvular aortoplasty (Brom’s procedure), single-patch augmentation of the non-coronary sinus and ascending aorta, and PDA ligation. Postoperative echocardiography demonstrated a peak gradient of 2–4 mmHg across the left ventricular outflow tract with no residual aortic regurgitation. The patient had an uneventful recovery. Conclusion: Single-patch supravalvular aortoplasty with PDA ligation is an effective and safe surgical strategy in children with SVAS, BAV, and anomalous coronary anatomy, providing relief of obstruction while preserving valve and coronary function.