Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

Taylor E Katt; Robert L Spicer; Anji T Yetman; Ali N Ibrahimiye; James M Hammel; Jeffrey A Robinson

doi:10.1016/j.jaccas.2020.05.098

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle

JACC Case Rep. 2020 Aug 12;2(11):1716-1719. doi: 10.1016/j.jaccas.2020.05.098. eCollection 2020 Sep.

Authors

Taylor E Katt¹, Robert L Spicer^{2

3}, Anji T Yetman^{2

3}, Ali N Ibrahimiye^{2

4}, James M Hammel^{2

4}, Jeffrey A Robinson^{2

3}

Affiliations

¹ College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska.
² Dr. C.C. and Mabel L. Criss Heart Center, Children's Hospital and Medical Center, Omaha, Nebraska.
³ Department of Pediatrics, Division of Cardiology, College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska.
⁴ Department of Surgery, College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska.

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: DOL, day of life; DORV, double-outlet right ventricle; LPA, left pulmonary artery; MPA, main pulmonary artery; POD, post-operative day; RPA, right pulmonary artery; RV, right ventricle; WS, Williams syndrome; aortic arch hypoplasia; congenital heart disease; genetic syndrome; pulmonary artery stenosis.

Publication types

Case Reports