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Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

Publication ,  Journal Article
Hornik, CP; Collins, RT; Jaquiss, RDB; Jacobs, JP; Jacobs, ML; Pasquali, SK; Wallace, AS; Hill, KD
Published in: J Thorac Cardiovasc Surg
June 2015

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.

Duke Scholars

Published In

J Thorac Cardiovasc Surg

DOI

EISSN

1097-685X

Publication Date

June 2015

Volume

149

Issue

6

Start / End Page

1516 / 22.e1

Location

United States

Related Subject Headings

  • Williams Syndrome
  • Treatment Outcome
  • Time Factors
  • Societies, Medical
  • Risk Factors
  • Retrospective Studies
  • Respiratory System
  • Registries
  • Postoperative Complications
  • Odds Ratio
 

Citation

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Hornik, C. P., Collins, R. T., Jaquiss, R. D. B., Jacobs, J. P., Jacobs, M. L., Pasquali, S. K., … Hill, K. D. (2015). Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg, 149(6), 1516-22.e1. https://doi.org/10.1016/j.jtcvs.2015.02.016
Hornik, Christoph P., Ronnie Thomas Collins, Robert D. B. Jaquiss, Jeffrey P. Jacobs, Marshall L. Jacobs, Sara K. Pasquali, Amelia S. Wallace, and Kevin D. Hill. “Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.J Thorac Cardiovasc Surg 149, no. 6 (June 2015): 1516-22.e1. https://doi.org/10.1016/j.jtcvs.2015.02.016.
Hornik CP, Collins RT, Jaquiss RDB, Jacobs JP, Jacobs ML, Pasquali SK, et al. Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 2015 Jun;149(6):1516-22.e1.
Hornik, Christoph P., et al. “Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.J Thorac Cardiovasc Surg, vol. 149, no. 6, June 2015, pp. 1516-22.e1. Pubmed, doi:10.1016/j.jtcvs.2015.02.016.
Hornik CP, Collins RT, Jaquiss RDB, Jacobs JP, Jacobs ML, Pasquali SK, Wallace AS, Hill KD. Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery: An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 2015 Jun;149(6):1516–22.e1.
Journal cover image

Published In

J Thorac Cardiovasc Surg

DOI

EISSN

1097-685X

Publication Date

June 2015

Volume

149

Issue

6

Start / End Page

1516 / 22.e1

Location

United States

Related Subject Headings

  • Williams Syndrome
  • Treatment Outcome
  • Time Factors
  • Societies, Medical
  • Risk Factors
  • Retrospective Studies
  • Respiratory System
  • Registries
  • Postoperative Complications
  • Odds Ratio