Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

Abstract

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.)

Keywords

• aortic arch hypoplasia
• congenital heart disease
• genetic syndrome
• pulmonary artery stenosis