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1 results

  • Chapter 17 - Supravalvular Aortic Stenosis

  • from Section 3 - Left-Sided Obstructive Lesions
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 105-112

  • 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.