Supravalvulaire Aortic Stenosis: Case Report

Introduction: Exceptional congenital heart disease (1 for 26000 birth) characterized by rétrécissement of aortic light. It can be isolated or part of William syndrome. The diagnosis is based on echocardiography. The intervention consists of an aortic root enlargement with Dacron patch. Surgery was indicated if gradient aortic left ventricle Superior of 50 mm hg. This clinical case is for us an opportunity to recall of this type of congenital aortic retrécissement. Methods: We reported the observation of patient 17-year-old without history presented since one-year dyspnea on exersion, palpitation and syncopes. Physical examination: murmur systolic in aortic home without other abnormalities. Chest X ray: CTI: 0.48., ECG: RSR with HVL. Echocardiography: supravalvulaire aortic stenosis; mean gradient AO-LV: 46 mm hg, LV -aortic; LV 48/26 mm + HLV, RV: 20 mm. Exploration per-opératoire: hipoplasie of the left coronary sinus, anomaly of implantation of antero -external pillar of mitral valve, aortic bicuspidie type 1, absence of coronary anomalies. It has benefit under cardio-pulmonary bypass an enlargement of the left coronary sinus according to DOTY technique with Dacron patch and conservation of aortic valve. Results: The immediate post-operative suites were favoured with gradient aortic –left ventricle drop to 20 mm hg. Conclusion: This is very rare congenital heart disease; echocardiography remains the key of diagnosis. It must be operated early. The prognostic is enhanced by the advances in surgical techniques. The treatment consists of surgery.