Open Access

REVIEW

Peri-procedural risk stratification and management of patients with Williams syndrome

R. Thomas Collins II^1,2, Margaret G. Collins³, Michael L. Schmitz^2,4, Justin T. Hamrick⁴

1 Department of Pediatrics, The University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
2 Department of Internal Medicine, The University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
3 Department of Dentistry, Arkansas Children’s Hospital, Little Rock, Arkansas, USA
4 Department of Anesthesiology, The University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA

* Corresponding Author: R. Thomas Collins, Arkansas Children’s Hospital, 1 Children’s Way, Mail Slot 512-3, Little Rock, AR 72202, USA. Email:

Congenital Heart Disease 2017, 12(2), 133-142. https://doi.org/10.1111/chd.12447

Abstract

Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. Sudden cardiovascular collapse in patients with WS is a well-known phenomenon, especially in the peri-procedural period. Detailed guidelines for peri-procedural management of patients with WS are limited. The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS with careful consideration of hemodynamic impacts of anesthetic strategies. In addition, an expanded risk stratification system for anesthetic administration is provided.

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Keywords

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