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Sudden cardiac death associated with cardiac catheterization in Williams syndrome: a case report and review of literature

Published online by Cambridge University Press:  05 April 2019

Samer Abu-Sultaneh Open the ORCID record for Samer Abu-Sultaneh [Opens in a new window] ,
Mercia J. Gondim ,
Ryan D. Alexy  and
Christopher W. Mastropietro
Samer Abu-Sultaneh*
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care Medicine, Indiana University School of Medicine, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Mercia J. Gondim
Affiliation:
Department of Pathology & Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
Ryan D. Alexy
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine and Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
Christopher W. Mastropietro
Affiliation:
Department of Pediatrics, Division of Pediatric Critical Care Medicine, Indiana University School of Medicine, Riley Hospital for Children at Indiana University Health, Indianapolis, IN, USA
*
Author for correspondence: Samer Abu-Sultaneh, MD, FAAP, Pediatric Critical Care, Riley Hospital for Children at Indiana University Health, 705 Riley Hospital Drive, Riley Phase 2 Room 4900, Indianapolis, IN 46202-5225, USA. Tel: +1 317 948 7185; Fax: +1 317 944 7267; E-mail: [email protected]
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Abstract

Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Keywords

Williams syndromepaediatricscardiac catheterisationdeathsuddencardiaccoronary stenosisanaesthesiageneral
Type
Review Article
Information
Cardiology in the Young , Volume 29 , Issue 4 , April 2019 , pp. 457 - 461
Copyright
© Cambridge University Press 2019 

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