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Chapter 17 - Supravalvular Aortic Stenosis

from Section 3 - Left-Sided Obstructive Lesions

Published online by Cambridge University Press:  09 September 2021

By
Stephanie N. Grant  and
Bruce E. Miller
Edited by
Laura K. Berenstain  and
James P. Spaeth
Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

Williams syndrome is a multisystem disorder with a variable clinical presentation resulting from a chromosome 7q11.23 microdeletion including the ELN gene coding for the protein elastin. Eighty percent of patients have cardiovascular abnormalities including supravalvar aortic stenosis, branch and peripheral pulmonary artery stenosis, and coronary artery obstructions. Patients are at high risk for cardiac arrest when undergoing sedation or general anesthesia due to an imbalance in myocardial oxygen supply and demand induced by anesthetic medications. Risk factors for cardiac arrest in the perioperative period are biventricular outflow tract obstruction and coronary artery obstruction by ostial or intraluminal stenosis. Hemodynamic goals include maintaining an age-appropriate heart rate, sinus rhythm, preload, contractility, and systemic vascular resistance. Sedation or general anesthesia for elective procedures should be performed at tertiary care medical centers with immediate access to cardiac specialists and the capability to provide extracorporeal membrane oxygenation support.

Keywords

cardiac arrestcoronary artery stenosiselastin arteriopathysudden deathsupravalvular aortic stenosisWilliams syndromeWilliams–Beuren syndrome
Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
 , pp. 105 - 112
Publisher: Cambridge University Press
Print publication year: 2021

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References

References

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Suggested Reading

Brown, M. L., Nasr, V. G., Toohey, R., et al. Williams syndrome and anesthesia for non-cardiac surgery: high risk can be mitigated with appropriate planning. Pediatr Cardiol 2018; 39: 1123–28.CrossRefGoogle ScholarPubMed
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Collins, R. T., Collins, M. G., Schmitz, M. L., et al. Peri-procedural risk stratification and management of patients with Williams syndrome. Congenit Heart Dis 2017; 12: 133–42.CrossRefGoogle Scholar
Latham, G. J., Ross, F. J., Eisses, M. J., et al. Perioperative morbidity in children with elastin arteriopathy. Pediatr Anesth 2016; 26: 926–35.CrossRefGoogle ScholarPubMed
Matisoff, A. J., Olivieri, L., Schwartz, J. M., et al. Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review. Pediatr Anesth 2015; 25: 1207–15.CrossRefGoogle ScholarPubMed
Pober, B. R. Williams-Beuren syndrome. N Engl J Med 2010; 362: 239–52.CrossRefGoogle ScholarPubMed
Twite, M. D., Stenquist, S., and Ing, R. J. Williams syndrome. Pediatr Anesth 2019; 29: 483–90.CrossRefGoogle ScholarPubMed

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