Kenyon Christine A, Flick Randall, Moir Christopher, Ackerman Michael J, Pabelick Christina M
Department of Anesthesiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Paediatr Anaesth. 2010 May;20(5):465-70. doi: 10.1111/j.1460-9592.2010.03293.x. Epub 2010 Mar 22.
To describe our experience in the anesthetic management of pediatric patients who have undergone left cardiac sympathetic denervation (LCSD) for congenital long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT).
Long QT syndrome and CPVT predispose patients to ventricular arrhythmias and sudden death. One treatment option for these patients is LCSD. When these patients present for LCSD or other surgical procedures, anesthetic management is challenging, as many medications may exacerbate QT prolongation.
Retrospective review of the electronic medical records of 22 pediatric patients who underwent LCSD between November 2005 and December 2008.
Six patients (27%) received midazolam as a premedication. Eleven patients (50%) underwent inhalation induction with sevoflurane. Eighty-six percentage received either sevoflurane or isoflurane for maintenance of anesthesia, while the remaining 14% received a propofol infusion. Nine patients (41%) received esmolol infusions intraoperatively, while one patient (4.5%) received a labetalol infusion. Three patients (14%) received lidocaine infusions. no significant cardiac or other events occurred in any of these patients in the perioperative period.
Important anesthetic considerations in this population include avoidance of sympathetic stimulation, correction of any abnormal electrolytes, and the immediate availability of a defibrillator and magnesium sulfate to treat arrhythmias. Anxious patients may benefit from premedication to reduce sympathetic tone. We have safely used both volatile agents and propofol for induction and maintenance of anesthesia. In our experience, intraoperative infusions of beta-blockers and lidocaine seem to be helpful in reducing arrhythmogenic potential, especially in patients with profound QT prolongation.
描述我们对因先天性长QT综合征（LQTS）和儿茶酚胺能多形性室性心动过速（CPVT）而接受左心交感神经去神经术（LCSD）的儿科患者进行麻醉管理的经验。
长QT综合征和CPVT使患者易患室性心律失常和猝死。这些患者的一种治疗选择是LCSD。当这些患者因LCSD或其他外科手术就诊时，麻醉管理具有挑战性，因为许多药物可能会加重QT间期延长。
回顾性分析2005年11月至2008年12月期间接受LCSD的22例儿科患者的电子病历。
6例患者（27%）术前使用咪达唑仑。11例患者（50%）采用七氟醚吸入诱导。86%的患者在麻醉维持期使用七氟醚或异氟醚，其余14%的患者接受丙泊酚输注。9例患者（41%）术中接受艾司洛尔输注，1例患者（4.5%）接受拉贝洛尔输注。3例患者（14%）接受利多卡因输注。这些患者在围手术期均未发生显著的心脏或其他事件。
该人群重要的麻醉注意事项包括避免交感神经刺激、纠正任何异常电解质以及随时准备好除颤器和硫酸镁以治疗心律失常。焦虑的患者可能从术前用药中受益，以降低交感神经张力。我们已安全地使用挥发性麻醉剂和丙泊酚进行麻醉诱导和维持。根据我们的经验，术中输注β受体阻滞剂和利多卡因似乎有助于降低致心律失常的可能性，尤其是在QT间期显著延长的患者中。
