PDF(Pubmed)
Abstract：
Andersen-Tawil syndrome (ATS) is a rare genetic disease characterized by a triad of episodic flaccid muscle weakness, ventricular arrhythmias, and physical anomalies. ATS patients have various cardiac arrhythmias that can cause sudden death. Implantation of an implantable cardioverter-defibrillator (ICD) is required when life-threatening cardiac arrhythmias do not respond to medical treatment. An 11-year-old girl underwent surgery for an ICD implantation. For general anesthesia in ATS patients, anesthesiologists should focus on the potentially difficult airway, serious cardiac arrhythmias, such as ventricular tachycardia (VT), and delayed recovery from neuromuscular blockade. We followed the difficult airway algorithm, avoided drugs that can precipitate QT prolongation and fatal cardiac arrhythmias, and tried to maintain normoxia, normocarbia, normothermia, normoglycemia, and pain control for prevention of sympathetic stimulation. We report the successful application of general anesthesia for ICD implantation in a pediatric patient with ATS and recurrent VT.
摘要：
Andersen-Tawil综合征（ATS）是一种罕见的遗传性疾病，其特征是发作性弛缓性肌肉无力的三联症，室性心律失常,和物理异常。ATS患者有各种心律失常，可导致猝死。当危及生命的心律失常对药物治疗无反应时，需要植入植入式心脏复律除颤器（ICD）。一名11岁的女孩接受了ICD植入手术。对于ATS患者的全身麻醉，麻醉师应该关注潜在的困难气道，严重的心律失常,例如室性心动过速（VT），神经肌肉阻滞的恢复延迟。我们遵循了困难的气道算法，避免可能导致QT延长和致命心律失常的药物，并试图保持常氧，normocarbia,体温正常,血糖正常,和预防交感神经刺激的疼痛控制。我们报告了在患有ATS和复发性VT的儿科患者中，全身麻醉在ICD植入中的成功应用。
