Key result
Anaesthetic management avoiding sympathetic stimulation and QT-prolonging drugs allowed for an uneventful bilateral thoracoscopic cervicothoracic sympathectomy in a 3-year-old boy with Jervell and Lange-Nielsen syndrome.
Case Report (n=1)
No
This case report highlights the anaesthetic considerations for a child with Jervell and Lange-Nielsen syndrome undergoing cervicothoracic sympathectomy to manage arrhythmogenic risk.
Long QT syndrome (LQTS) is an arrhythmogenic cardiac disorder resulting from the malfunction of cardiac ion channels. Patient with LQTS may present with syncope, seizures or sudden cardiac death secondary to polymorphic ventricular tachycardia (VT) or torsades de pointes. Patient may be asymptomatic in the pre-operative period but may develop VT for the first time in operation theatre. We are reporting anaesthetic management of a child with LQTS planned for bilateral thoracoscopic cervicothoracic sympathectomy.
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Roy et al. (2016) conducted a case report in Jervell and Lange-Nielsen syndrome (Long QT syndrome) (n=1). Anaesthetic management for bilateral thoracoscopic cervicothoracic sympathectomy was evaluated on Successful surgery without major intraoperative cardiac events. Anaesthetic management avoiding sympathetic stimulation and QT-prolonging drugs allowed for an uneventful bilateral thoracoscopic cervicothoracic sympathectomy in a 3-year-old boy with Jervell and Lange-Nielsen syndrome.
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