{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T02:02:34Z","timestamp":1781056954726,"version":"3.54.1"},"reference-count":9,"publisher":"BMJ","issue":"6","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,2,20]]},"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:p>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\u2013T1), including the lower stellate, was completed uneventfully. The child was extubated in the operating room and discharged the next day without complications.<\/jats:p>","DOI":"10.1136\/bcr-2025-271298","type":"journal-article","created":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T01:55:13Z","timestamp":1781056513000},"page":"e271298","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Multidisciplinary management of video-assisted thoracoscopic left cardiac sympathetic denervation in a young child with Jervell and Lange-Nielsen syndrome"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7565-869X","authenticated-orcid":false,"given":"Muhammad Saad","family":"Yousuf","sequence":"first","affiliation":[{"name":"Anaesthesia, The Aga Khan University Hospital, Karachi, Sindh, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khalid","family":"Samad","sequence":"additional","affiliation":[{"name":"Anaesthesia, The Aga Khan University Hospital, Karachi, Sindh, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad Hassan","family":"Gazdar","sequence":"additional","affiliation":[{"name":"Anaesthesia, The Aga Khan University Hospital, Karachi, Sindh, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad","family":"Mohsin","sequence":"additional","affiliation":[{"name":"Cardiology, National Institute of Cardiovascular Diseases, Karachi, Sindh, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saleem","family":"Islam","sequence":"additional","affiliation":[{"name":"Surgery, The Aga Khan University Hospital, Karachi, Sindh, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,6,9]]},"reference":[{"key":"2026060918550622000_19.6.e271298.1","doi-asserted-by":"crossref","DOI":"10.1093\/europace\/euae136","article-title":"Genetic characterization of KCNQ1 variants improves risk stratification in type 1 long QT syndrome patients","volume":"26","author":"Morgat","year":"2024","journal-title":"Europace"},{"key":"2026060918550622000_19.6.e271298.2","doi-asserted-by":"crossref","DOI":"10.1155\/2020\/3569359","article-title":"Jervell and Lange-Nielsen Syndrome due to a Novel Compound Heterozygous KCNQ1 Mutation in a Chinese Family","volume":"2020","author":"Qiu","year":"2020","journal-title":"Neural Plast"},{"key":"2026060918550622000_19.6.e271298.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2013.07.064"},{"key":"2026060918550622000_19.6.e271298.4","doi-asserted-by":"crossref","first-page":"2186","DOI":"10.1016\/j.hrthm.2016.07.020","article-title":"The Jervell and Lange-Nielsen syndrome; atrial pacing combined with \u00df-blocker therapy, a favorable approach in young high-risk patients with long QT syndrome?","volume":"13","author":"Fr\u00fch","year":"2016","journal-title":"Heart Rhythm"},{"key":"2026060918550622000_19.6.e271298.5","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.015731"},{"key":"2026060918550622000_19.6.e271298.6","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1111\/anae.15609","article-title":"PROSPECT guidelines for video-assisted thoracoscopic surgery: a systematic review and procedure-specific postoperative pain management recommendations","volume":"77","author":"Feray","year":"2022","journal-title":"Anaesthesia"},{"key":"2026060918550622000_19.6.e271298.7","doi-asserted-by":"crossref","first-page":"2025277","DOI":"10.31893\/multirev.2025277","article-title":"Anesthesia in Long QT syndrome: A review of pathophysiology, risk factors, and management strategies","volume":"8","author":"Dhanure","year":"2025","journal-title":"Multidiscip Rev"},{"key":"2026060918550622000_19.6.e271298.8","doi-asserted-by":"publisher","DOI":"10.1213\/ANE.0000000000000389"},{"key":"2026060918550622000_19.6.e271298.9","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/j.jacep.2021.09.002","article-title":"Left Cardiac Sympathetic Denervation for Long QT Syndrome: 50 Years\u2019 Experience Provides Guidance for Management","volume":"8","author":"Dusi","year":"2022","journal-title":"JACC Clin Electrophysiol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2025-271298","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T01:55:18Z","timestamp":1781056518000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2025-271298"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6]]},"references-count":9,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2026,6,9]]},"published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1136\/bcr-2025-271298"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2025-271298","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6]]}}}