左心室流出道梯度与患有威廉姆斯-贝伦综合征的儿童的冠状动脉阻塞有关。
Left Ventricular Outflow Tract Gradient Is Associated With Coronary Artery Obstruction in Children With Williams-Beuren Syndrome.
机构信息
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.
Department of Cardiology, Boston Children's Hospital, Boston, MA; Department of Pediatrics, Harvard Medical School, Boston, MA.
出版信息
J Cardiothorac Vasc Anesth. 2021 Dec;35(12):3677-3680. doi: 10.1053/j.jvca.2020.12.050. Epub 2021 Jan 4.
OBJECTIVES
Patients 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.
DESIGN
Retrospective 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.
SETTING
Single-center university teaching hospital.
PARTICIPANTS
The study included 49 patients with Williams-Beuren syndrome who underwent transthoracic echocardiogram, cardiac catheterization, and/or surgical interventions.
MEASUREMENTS AND MAIN RESULTS
The 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).
CONCLUSION
LVOT gradient can serve as a good predictor of the presence of coronary obstruction in patients with Williams-Beuren syndrome.
目的
患有威廉姆斯-贝伦综合征的患者在镇静和/或麻醉期间存在血流动力学崩溃的高风险，推测这是由于隐匿性冠状动脉阻塞所致。本研究的目的是确定经胸超声心动图检查结果与冠状动脉阻塞之间的关系，以检查冠状动脉阻塞是否可以通过麻醉前的经胸超声心动图来预测。
设计
对患有威廉姆斯-贝伦综合征的患者进行回顾性数据分析，这些患者接受了经胸超声心动图、心导管检查和/或手术干预，以确定超声心动图检查结果与心导管检查和/或手术确定的冠状动脉阻塞之间的相关性。
地点
单中心大学教学医院。
参与者
该研究纳入了 49 名接受经胸超声心动图、心导管检查和/或手术干预的威廉姆斯-贝伦综合征患者。
测量和主要结果
唯一与冠状动脉阻塞相关的变量是经胸超声心动图上左心室流出道（LVOT）的最大瞬时梯度（MIG）。LVOT MIG≥75mmHg 作为最佳截断值与冠状动脉阻塞相关（曲线下面积 0.659，优势比 6.71，95%可信区间 1.31-34.35，p=0.022）。
结论
LVOT 梯度可以作为预测威廉姆斯-贝伦综合征患者冠状动脉阻塞存在的良好指标。
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