title: Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.
abstract: OBJECTIVE
  Patients with Williams syndrome (WS) undergoing cardiac surgery are at risk for major adverse cardiac events (MACE). Prevalence and risk factors for such events have not been well described. We sought to define frequency and risk of MACE in patients with WS using a multicenter clinical registry.
  
  
  METHODS
  We identified cardiac operations performed in patients with WS using the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000-2012). Operations were divided into 4 groups: isolated supravalvular aortic stenosis, complex left ventricular outflow tract (LVOT), isolated right ventricular outflow tract (RVOT), and combined LVOT/RVOT procedures. The proportion of patients with MACE (in-hospital mortality, cardiac arrest, or postoperative mechanical circulatory support) was described and the association with preoperative factors was examined.
  
  
  RESULTS
  Of 447 index operations (87 centers), median (interquartile range) age and weight at surgery were 2.4 years (0.6-7.4 years) and 10.6 kg (6.5-21.5 kg), respectively. Mortality occurred in 20 patients (5%). MACE occurred in 41 patients (9%), most commonly after combined LVOT/RVOT (18 out of 87; 21%) and complex LVOT (12 out of 131; 9%) procedures, but not after isolated RVOT procedures. Odds of MACE decreased with age (odds ratio [OR], 0.99; 95% confidence interval [CI], 0.98-0.99), weight (OR, 0.97; 95% CI, 0.93-0.99), but increased in the presence of any preoperative risk factor (OR, 2.08; 95% CI, 1.06-4.00), and in procedures involving coronary artery repair (OR, 5.37; 95% CI, 2.05-14.06).
  
  
  CONCLUSIONS
  In this multicenter analysis, MACE occurred in 9% of patients with WS undergoing cardiac surgery. Demographic and operative characteristics were associated with risk. Further study is needed to elucidate mechanisms of MACE in this high-risk population.
authors: Department of Pediatrics, Duke University, Durham, NC; Duke Clinical Research Institute, Duke University, Durham, NC. Electronic address: christoph.hornik@dm.duke.edu.
authors: Christoph P
authors: CP
authors: Hornik
authors: Division of Pediatric Cardiology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, Ark.
authors: Ronnie Thomas
authors: RT
authors: Collins
authors: Department of Pediatrics, Duke University, Durham, NC; Duke Clinical Research Institute, Duke University, Durham, NC.
authors: Robert D B
authors: RD
authors: Jaquiss
authors: Andrews/Daicoff Cardiovascular Program, Johns Hopkins Children's Heart Surgery, St Petersburg, Fla; All Children's Hospital, Tampa, Fla; Florida Hospital for Children, Orlando, Fla.
authors: Jeffrey P
authors: JP
authors: Jacobs
authors: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Md.
authors: Marshall L
authors: ML
authors: Jacobs
authors: Division of Pediatric Cardiology, University of Michigan C.S. Mott Children's Hospital, Ann Arbor, Mich.
authors: Sara K
authors: SK
authors: Pasquali
authors: Department of Pediatrics, Duke University, Durham, NC; Duke Clinical Research Institute, Duke University, Durham, NC.
authors: Amelia S
authors: AS
authors: Wallace
authors: Department of Pediatrics, Duke University, Durham, NC; Duke Clinical Research Institute, Duke University, Durham, NC.
authors: Kevin D
authors: KD
authors: Hill
mesh_terms: D000367:Age Factors; D006348:Cardiac Surgical Procedures / Q000009:adverse effects* / Q000401:mortality; D016009:Chi-Square Distribution; D046688:Child Mortality; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D006323:Heart Arrest / Q000209:etiology; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D015994:Incidence; D007223:Infant; D007226:Infant Mortality; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D009656:North America / Q000453:epidemiology; D016017:Odds Ratio; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D012955:Societies, Medical; D013997:Time Factors; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000601:surgery*
keywords: Williams syndrome; major adverse cardiac events; risk factors