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Case reports

Management of supravalvar aortic stenosis and severely depressed left ventricular function in a neonate with Williams syndrome

^a Division of Cardiac Surgery, Department of Surgery, Montreal Children's Hospital, McGill University, Montreal, Canada
^b Department of Pediatric Cardiology, Montreal Children's Hospital, McGill University, Montreal, Canada

Received 9 November 2008; received in revised form 7 January 2009; accepted 12 January 2009.

^_* Corresponding author. Address: Division of Cardiac Surgery, Department of Surgery, McGill University Health Center, Montreal Children's Hospital, 2300 Tupper Street, Room C-827, Montreal, PQ H3H 1P3, Canada. Tel.: +1 514 934 4400; fax: +1 514 934 4368. (Email: dr_turki{at}yahoo.com).

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.

Key Words: Supravalvar aortic stenosis • Aortic arch obstruction • Neonate • Williams syndrome