• Alexander R Schmidt, R Thomas Collins, Yamini Adusumelli, Chandra Ramamoorthy, Yingjie Weng, Kirstie L MacMillen, and Manchula Navaratnam.
• Division of Pediatric Cardiac Anesthesia, Lucile Packard Children's Hospital Stanford and Stanford University School of Medicine, Palo Alto, CA. Electronic address: scale@stanford.edu.
• J. Cardiothorac. Vasc. Anesth. 2021 Dec 1; 35 (12): 3667-3674.

ObjectiveThis study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy.DesignRetrospective observational cohort study.SettingSingle quaternary academic referral center.ParticipantsThe authors reviewed the records of all children with WS at the authors' institution who underwent general anesthesia for cardiac catheterization, diagnostic imaging, or any type of surgery between November 2008 and August 2019. The authors identified 142 patients with WS, 48 of whom underwent 118 general anesthesia administrations. A historic group (HG) was compared with the intervention group (IG).InterventionsChange in perioperative management (three-stage risk stratification: preoperative intravenous hydration, intravenous anesthesia induction, and early use of vasoactives).Measurements And Main ResultsThe authors determined event rates within 60 minutes of anesthesia induction. Standardized mean difference (SMD) was calculated (SMD >0.2 suggests clinically meaningful difference). Sixty-seven general anesthesia encounters were recorded in the HG (mean age, 4.8 years; mean weight, 16.3 kg) and 51 in the IG (mean age, 6.0 years; mean weight, 18.2 kg). The change in systolic blood pressure was -17.5% (-30.0, -5.0) in the HG versus -9% (-18.0, 5.0) in the IG (p = 0.015; SMD = 0.419), and the incidence of ACEs was 6% in the HG and 2% in the IG (p = 0.542; SMD = 0.207).ConclusionsPreoperative risk stratification, preoperative intravenous hydration, intravenous induction, and early use of continuous vasoactives resulted in greater hemodynamic stability, with a 2% incidence of ACEs.Copyright © 2021 Elsevier Inc. All rights reserved.

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