Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery

An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database

Christoph P. Hornik, Ronnie Thomas Collins, Robert D B Jaquiss, Jeffrey P. Jacobs, Marshall L. Jacobs, Sara K. Pasquali, Amelia S. Wallace, Kevin D. Hill

Research output: Contribution to journalArticle

23 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1516-1522e1
JournalJournal of Thoracic and Cardiovascular Surgery
Volume149
Issue number6
DOIs
StatePublished - Jan 1 2015

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Williams Syndrome
Thoracic Surgery
Databases
Odds Ratio
Confidence Intervals
Supravalvular Aortic Stenosis
Weights and Measures
Hospital Mortality
Heart Arrest
Registries
Coronary Vessels
Demography
Mortality

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine
  • Medicine(all)

Cite this

Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery : An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. / Hornik, Christoph P.; Collins, Ronnie Thomas; Jaquiss, Robert D B; Jacobs, Jeffrey P.; Jacobs, Marshall L.; Pasquali, Sara K.; Wallace, Amelia S.; Hill, Kevin D.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 149, No. 6, 01.01.2015, p. 1516-1522e1.

Research output: Contribution to journalArticle

Hornik, Christoph P. ; Collins, Ronnie Thomas ; Jaquiss, Robert D B ; Jacobs, Jeffrey P. ; Jacobs, Marshall L. ; Pasquali, Sara K. ; Wallace, Amelia S. ; Hill, Kevin D. / Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery : An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. In: Journal of Thoracic and Cardiovascular Surgery. 2015 ; Vol. 149, No. 6. pp. 1516-1522e1.
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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.",
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T1 - Adverse cardiac events in children with Williams syndrome undergoing cardiovascular surgery

T2 - An analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database

AU - Hornik, Christoph P.

AU - Collins, Ronnie Thomas

AU - Jaquiss, Robert D B

AU - Jacobs, Jeffrey P.

AU - Jacobs, Marshall L.

AU - Pasquali, Sara K.

AU - Wallace, Amelia S.

AU - Hill, Kevin D.

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N2 - 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.

AB - 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.

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