Scott-Warren Victoria, Bendon Anju, Bruce Iain A, Henderson Lise, Diacono Jacques
a Department of Paediatric Anaesthesia , Royal Manchester Children's Hospital , Manchester , UK.
b Paediatric ENT Department , Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre , Manchester , UK.
Cochlear Implants Int. 2018 Nov;19(6):350-354. doi: 10.1080/14670100.2018.1518686. Epub 2018 Sep 19.
Congenital long QT syndrome (cLQTS) is an inherited cardiac ion channelopathy characterized by a long corrected-QT interval on the ECG, associated with a risk of syncope and sudden death as a result of arrhythmias. The archetypal arrhythmia associated with cLQTS is torsade de pointes which may degenerate into ventricular fibrillation. Children with Jervell and Lange-Neilsen syndrome have the combination of cLQTS and congenital sensorineural deafness and may present for cochlear implantation (CI). Sympathetic stimulation and administration of QT-prolonging medications may trigger arrhythmias in children with cLQTS and thus the perioperative period is a time of increased risk of adverse events, with deaths reported in the CI literature. Our Paediatric Cochlear Implant Programme had previously elected to discontinue offering CI to children with cLQTS following a perioperative death. However, subsequent demand for this service by parents led us to develop and introduce a multidisciplinary, evidence-based pathway of care. This pathway modifies the perioperative management of these children to reduce the associated risk. We present the cases of four children with cLQTS who underwent CI in our specialist children's hospital.
先天性长QT综合征（cLQTS）是一种遗传性心脏离子通道病，其特征是心电图上校正QT间期延长，与心律失常导致的晕厥和猝死风险相关。与cLQTS相关的典型心律失常是尖端扭转型室速，可恶化为心室颤动。患有耶尔韦尔和朗格-尼尔森综合征的儿童兼具cLQTS和先天性感音神经性耳聋，可能需要进行人工耳蜗植入（CI）。交感神经刺激和使用延长QT间期的药物可能会触发cLQTS患儿的心律失常，因此围手术期是不良事件风险增加的时期，人工耳蜗植入文献中已有死亡报告。我们的儿科人工耳蜗植入项目此前在发生围手术期死亡后，决定不再为cLQTS患儿提供人工耳蜗植入服务。然而，随后家长对这项服务的需求促使我们制定并引入了一条多学科、循证的护理路径。这条路径对这些患儿的围手术期管理进行了调整，以降低相关风险。我们介绍了在我们的专科儿童医院接受人工耳蜗植入的4例cLQTS患儿的病例。
