• Angela Yun June Tan, Daniel Quiat, Sunil J Ghelani, and Koichi Yuki.
• Department of Anesthesiology, Critical Care and Pain Medicine, Cardiac Anesthesia Division, Boston Children's Hospital, Boston, MA; Department of Anesthesia, Harvard Medical School, Boston, MA; Department of Anesthesia, KK Women's and Children's Hospital, Singapore.
• J. Cardiothorac. Vasc. Anesth. 2021 Dec 1; 35 (12): 3677-3680.

ObjectivesPatients with Williams-Beuren syndrome are associated with a high risk of hemodynamic collapse during sedation and/or anesthesia, presumably due to occult coronary obstruction. The objective of this study was to determine the association between transthoracic echocardiogram findings and the presence of coronary obstruction to examine if coronary obstruction can be predicted by transthoracic echocardiogram before anesthesia.DesignRetrospective data analysis of patients with Williams-Beuren syndrome who underwent transthoracic echocardiogram, cardiac catheterization, and/or surgical interventions to determine the correlation between echocardiogram findings and the presence of coronary obstruction determined by cardiac catheterization and/or surgery.SettingSingle-center university teaching hospital.ParticipantsThe study included 49 patients with Williams-Beuren syndrome who underwent transthoracic echocardiogram, cardiac catheterization, and/or surgical interventions.Measurements And Main ResultsThe only variable associated with coronary artery obstruction was the maximum instantaneous gradient (MIG) across the left ventricular outflow tract (LVOT) on a transthoracic echocardiogram. LVOT MIG ≥ 75 mmHg as the optimal cutoff value was associated with coronary artery obstruction (area under the curve 0.659, odds ratio 6.71, 95% CI 1.31-34.35, p = 0.022).ConclusionLVOT gradient can serve as a good predictor of the presence of coronary obstruction in patients with Williams-Beuren syndrome.Copyright © 2021 Elsevier Inc. All rights reserved.

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