Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome.
MS, Y., K, S., MH, G., M, M., & S, I. (2026). Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome.. BMJ case reports. https://doi.org/10.1136/bcr-2025-271298
MS Y, K S, MH G, M M, S I. Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome.. BMJ case reports. 2026; doi: 10.1136/bcr-2025-271298
MS Y, K S, MH G, et al. Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome.[J]. BMJ case reports. 2026. DOI: 10.1136/bcr-2025-271298.
@article{ms2026,
author = {Yousuf MS and Samad K and Gazdar MH and Mohsin M and Islam S},
title = {Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome.},
journal = {BMJ case reports},
year = {2026},
doi = {10.1136/bcr-2025-271298},
note = {PMID: 42264940},
}
TY - JOUR AU - Yousuf MS AU - Samad K AU - Gazdar MH AU - Mohsin M AU - Islam S TI - Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome. T2 - BMJ case reports PY - 2026 DO - 10.1136/bcr-2025-271298 AN - PMID:42264940 ER -
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.