Coleman Melissa, Imundo Jason R, Cortez Daniel, Cohen Mark H, Dhar Padmani, Dalal Priti G
Department of Anesthesiology and Perioperative Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA.
Department of Pediatrics, Division of Pediatric Cardiology, Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA.
Am J Case Rep. 2020 Oct 15;21:e925602. doi: 10.12659/AJCR.925602.
BACKGROUND Long QT syndrome (LQTS) is an arrhythmogenic heart condition that can be congenital or acquired. Prolonged ventricular repolarizations in individuals with the disorder can cause fatal arrhythmias. Abnormal functioning of cardiac ion channels leads to arrhythmias such as torsades de pointes (TdP) and may be triggered by stress or medications. Many medications used in the perioperative period are triggers for the arrythmia. CASE REPORT A 7-year-old patient with known congenital LQTS type 2 presented for bilateral myringotomy and tube placement. The patient was otherwise healthy and taking propranolol daily. Preoperative midazolam was administered for anxiolysis, and induction of anesthesia was uneventful. He sustained an episode of TdP immediately following general anesthetic induction after failure of an in situ automatic implantable cardioverter-defibrillator (AICD). External defibrillation succeeded, and the patient was stabilized in the Postanesthesia Recovery Unit before transfer to the Pediatric Intensive Care Unit. Interrogation of the AICD revealed several undelivered defibrillation attempts. A chest X-ray showed an area suggestive of an epicardial electrode fracture. The following day, the AICD was replaced with no arrythmias noted. The patient had an uneventful recovery. CONCLUSIONS In patients with a known history of LQTS, preparation and prevention are cornerstones of anesthesia care. Minimizing the use of triggering medications and emotional stress in the perioperative period, combined with ready equipment and medications to respond to arrythmias, are essential. In children, there is a greater chance of lead fracture and resulting device failure. Preoperative history of device function or interrogation of the AICD and possibly a chest X-ray are essential to ensure the integrity of the leads.
长QT综合征（LQTS）是一种可先天性或后天获得的致心律失常性心脏病。该疾病患者的心室复极延长可导致致命性心律失常。心脏离子通道功能异常会导致诸如尖端扭转型室速（TdP）等心律失常，并且可能由压力或药物引发。围手术期使用的许多药物都是心律失常的触发因素。
一名已知患有2型先天性LQTS的7岁患者前来接受双侧鼓膜切开置管术。该患者其他方面健康，每天服用普萘洛尔。术前给予咪达唑仑进行镇静，麻醉诱导过程顺利。在植入式自动心脏复律除颤器（AICD）原位除颤失败后，全身麻醉诱导后他立即发生了一次TdP发作。体外除颤成功，患者在麻醉后恢复室稳定后转至儿科重症监护病房。对AICD的问询显示有几次未成功的除颤尝试。胸部X线片显示一个提示心外膜电极断裂的区域。第二天，更换了AICD，未发现心律失常。患者恢复顺利。
对于有LQTS病史的患者，准备和预防是麻醉护理的基石。在围手术期尽量减少触发药物的使用和情绪压力，同时准备好应对心律失常的设备和药物至关重要。在儿童中，导线断裂及由此导致设备故障的可能性更大。术前了解设备功能的病史或对AICD进行问询以及可能进行胸部X线检查对于确保导线的完整性至关重要。
