{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T00:55:46Z","timestamp":1784336146370,"version":"3.55.0"},"reference-count":16,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T00:00:00Z","timestamp":1695168000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pacing Clinical Electrophis"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Long QT syndrome is an inherited malignant channelopathy which leads to life\u2010threatening arrhythmia, with multiple genotypes. Jervell and Lange\u2010Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methodology<\/jats:title><jats:p>We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high\u2010risk features despite being on maximally tolerated doses of oral propranolol.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Mean age at diagnosis was 2.75\u00a0\u00b1\u00a00.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2\u00a0\u00b1\u00a03.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603\u00a0\u00b1\u00a093\u00a0ms, with T wave alternans (TWA) seen in all cases. All were started on propranolol and subsequently subjected to LCSD, and 3 underwent AAI permanent pacemaker implantation. Over a mean follow\u2010up of 20 months, there was a significant reduction in QTc (603\u00a0\u00b1\u00a093\u00a0ms to 501\u00a0\u00b1\u00a033\u00a0ms, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.04), which was persistent on follow\u2010up (525\u00a0\u00b1\u00a041\u00a0ms) and only two out of six had persistent T wave alternans on ECG (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Jervell Lange\u2010Nielsen syndrome is a subtype of LQTS with high\u2010risk features. LCSD, an effective therapeutic option for those having symptoms despite being on propranolol, results in significant reduction of QTc interval and amelioration of symptoms.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pace.14827","type":"journal-article","created":{"date-parts":[[2023,9,20]],"date-time":"2023-09-20T12:33:47Z","timestamp":1695213227000},"page":"1197-1202","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Left cardiac sympathetic denervation in children with Jervell Lange\u2010Nielsen syndrome and drug refractory torsades \u2013 A case series"],"prefix":"10.1111","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4823-7045","authenticated-orcid":false,"given":"Deepanjan","family":"Bhattacharya","sequence":"first","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narayanan","family":"Namboodiri","sequence":"additional","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madhusoodanan Pillai","family":"Sreelekshmi","sequence":"additional","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9908-2891","authenticated-orcid":false,"given":"Mukund A.","family":"Prabhu","sequence":"additional","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhilash","family":"Sreevilasam Pushpangadhan","sequence":"additional","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabarinath","family":"Menon","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular and Thoracic Surgery Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baiju S.","family":"Dharan","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular and Thoracic Surgery Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajitkumar","family":"Valaparambil","sequence":"additional","affiliation":[{"name":"Department of Cardiology Sree Chitra Tirunal Institute for Medical Sciences and Technology  Trivandrum 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