Management Of Supravalvar Aortic Stenosis And Severely Depressed Left Ventricular Function In A Neonate With Williams Syndrome

We report an interesting case of a patient with Williams syndrome who presented with moderate supravalvar aortic stenosis and bilateral pulmonary artery stenosis at one week of age. The supravalvar aortic stenosis became severe by the age of one month with severe depression of left ventricular function. The patient had a difficult postoperative course, developed an acquired aortic arch hypoplasia and required multiple interventions during the first two months of life with an excellent outcome. The management of this difficult patient is discussed with focus on the importance of close follow-up, early diagnosis and early surgical intervention in improving the outcome in this difficult group of patients. (C) 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.