Risk assessment and anesthetic management of patients with Williams syndrome: A comprehensive review

Andrew J. Matisoff, Laura Olivieri, Jamie Schwartz, Nina Deutsch

Research output: Contribution to journalArticle

Abstract

Since the first description in 1961, several case reports have documented an increased incidence of anesthesia-related cardiac arrest in patients with Williams-Beuren syndrome, commonly known as Williams syndrome (WS). Widespread arteriopathy secondary to an elastin gene defect results in various cardiac defects, including supravalvar aortic stenosis (SVAS) and coronary artery anomalies, which can increase the risk of myocardial ischemia. Even though patients with WS are known to have increased risk of adverse events during anesthesia and sedation, they often undergo several procedures that require anesthesia during their lifetimes, and cases of perianesthetic cardiac arrest continue to be reported. To date, no prospective studies have been reported that quantify anesthetic risk in individual patients with WS. In this article, we review the clinical manifestations of WS, propose a consensus, expert-informed method to estimate anesthetic risk based on the current literature, and provide recommendations for periprocedural management of this patient population.

Original languageEnglish (US)
Pages (from-to)1207-1215
Number of pages9
JournalPaediatric Anaesthesia
Volume25
Issue number12
DOIs
StatePublished - Dec 1 2015
Externally publishedYes

Fingerprint

Williams Syndrome
Anesthetics
Anesthesia
Heart Arrest
Supravalvular Aortic Stenosis
Elastin
Myocardial Ischemia
Coronary Vessels
Prospective Studies
Incidence
Population
Genes

Keywords

  • cardiac anesthesia
  • chromosome 7q11.23 deletion syndrome
  • perioperative medicine

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Risk assessment and anesthetic management of patients with Williams syndrome : A comprehensive review. / Matisoff, Andrew J.; Olivieri, Laura; Schwartz, Jamie; Deutsch, Nina.

In: Paediatric Anaesthesia, Vol. 25, No. 12, 01.12.2015, p. 1207-1215.

Research output: Contribution to journalArticle

Matisoff, Andrew J. ; Olivieri, Laura ; Schwartz, Jamie ; Deutsch, Nina. / Risk assessment and anesthetic management of patients with Williams syndrome : A comprehensive review. In: Paediatric Anaesthesia. 2015 ; Vol. 25, No. 12. pp. 1207-1215.
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