Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome

Left cardiac sympathetic denervation (LCSD) via video-assisted thoracoscopy (VATS) is an effective therapy for drug-refractory malignant arrhythmias in congenital long QT syndromes and requires meticulous perioperative planning in children with automatic implantable cardioverter-defibrillators (AICDs). We describe what is likely the first paediatric VATS-LCSD performed in Pakistan. A boy in early childhood with Jervell and Lange-Nielsen syndrome, severe QT prolongation and recurrent ventricular arrhythmias despite beta-blockade and mexiletine had received multiple AICD shocks. Intraoperative management focused on preventing electrocautery-induced AICD activation by applying a magnet to suspend antitachycardia therapies, using external defibrillation pads and ensuring continuous electrophysiology support. Anaesthesia incorporated sevoflurane, dexmedetomidine, cisatracurium and lidocaine, with one-lung ventilation achieved by intentional endobronchial tracheal tube placement. Thoracoscopic excision of the left sympathetic chain (T5–T1), including the lower stellate, was completed uneventfully. The child was extubated in the operating room and discharged the next day without complications.