Peri‐procedural risk stratification and management of patients with Williams syndrome. (March 2017)

• Record Type: Journal Article
• Authors: Collins II, R. Thomas; Collins, Margaret G.; Schmitz, Michael L.; Hamrick, Justin T.
• Is Part Of: Congenital heart disease. Volume 12:Number 2(2017)
• Page Start: 133
• Page End: 142
• Publication Date: 2017-03
• Subjects: anesthesia -- cardiac arrest -- dentistry -- perioperative care -- surgery -- Williams syndrome; Congenital heart disease -- Periodicals; 616.1204305
• DOI: 10.1111/chd.12447
• Languages: English
• ISSNs: 1747-079X

Abstract

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.