title: Perioperative morbidity in children with elastin arteriopathy.
abstract: BACKGROUND
  Children with elastin arteriopathy (EA), the majority of whom have Williams-Beuren syndrome, are at high risk for sudden death. Case reports suggest that the risk of perioperative cardiac arrest and death is high, but none have reported the frequency or risk factors for morbidity and mortality in an entire cohort of children with EA undergoing anesthesia.
  
  
  OBJECTIVE
  The aim of this study was to present one institution's rate of morbidity and mortality in all children with EA undergoing anesthesia and to examine patient characteristics that pose the greatest risk.
  
  
  METHODS
  We reviewed medical records of children with EA who underwent anesthesia or sedation for any procedure at our institution from 1990 to 2013. Cardiovascular hemodynamic indices from recent cardiac catheterization or echocardiography were tabulated for each child. The incidence, type, and associated factors of complications occurring intraoperatively through 48 h postoperatively were examined.
  
  
  RESULTS
  Forty-eight patients with confirmed EA underwent a total of 141 anesthetics. There were seven cardiac arrests (15% of patients, 5% of anesthetics) and nine additional intraoperative cardiovascular complications (15% of patients, 6% of anesthetics). Extracorporeal life support was initiated in five cases. There were no perioperative deaths. All children having a cardiac arrest or complication were <3 years old and had biventricular outflow tract obstruction (BVOTO). Subgroup analysis demonstrated high rates of cardiac arrest in two groups: children with BVOTO (44%) and age <3 years old (21%).
  
  
  CONCLUSIONS
  We have confirmed that the rate of cardiac arrest and complications is significantly elevated in children with EA undergoing anesthesia. Children <3 years old and with BVOTO were at the greatest risk in our population.
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Latham
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Faith J
authors.initials: FJ
authors.lastname: Ross
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Eisses
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Richards
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Jeremy M
authors.initials: JM
authors.lastname: Geiduschek
authors.affiliation: Department of Anesthesiology & Pain Medicine, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Denise C
authors.initials: DC
authors.lastname: Joffe
mesh_terms: D000758:Anesthesia; D002675:Child, Preschool; D015897:Comorbidity; D004549:Elastin; D005260:Female; D006323:Heart Arrest / Q000453:epidemiology*; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000453:epidemiology*; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D018980:Williams Syndrome / Q000453:epidemiology*
chemical_list: D004549:Elastin
keywords: Williams syndrome; Williams-Beuren syndrome; anesthesia; cardiac arrest; extracorporeal membrane oxygenation; pediatric; sudden cardiac death; supravalvar aortic stenosis syndrome