Left cardiac sympathetic denervation in children with Jervell Lange‐Nielsen syndrome and drug refractory torsades – A case series

Abstract Introduction Long QT syndrome is an inherited malignant channelopathy which leads to life‐threatening arrhythmia, with multiple genotypes. Jervell and Lange‐Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a hi...

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Published in:Pacing and clinical electrophysiology 2023-10, Vol.46 (10), p.1197-1202
Main Authors: Bhattacharya, Deepanjan, Namboodiri, Narayanan, Sreelekshmi, Madhusoodanan Pillai, Prabhu, Mukund A., Sreevilasam Pushpangadhan, Abhilash, Menon, Sabarinath, Dharan, Baiju S., Valaparambil, Ajitkumar
Format: Article
Language:English
Subjects:
Arrhythmia
Channelopathy
Children
Deafness
Denervation
Diagnosis
EKG
Genotypes
Hearing loss
Heart
Jervell and Lange-Nielsen syndrome
Long QT syndrome
Phenotypes
Propranolol
Seizures
Syncope
Torsades de pointes
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Summary:Abstract Introduction Long QT syndrome is an inherited malignant channelopathy which leads to life‐threatening arrhythmia, with multiple genotypes. Jervell and Lange‐Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD). Methodology We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high‐risk features despite being on maximally tolerated doses of oral propranolol. Results Mean age at diagnosis was 2.75 ± 0.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2 ± 3.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603 ± 93 ms, 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‐up of 20 months, there was a significant reduction in QTc (603 ± 93 ms to 501 ± 33 ms, p  = .04), which was persistent on follow‐up (525 ± 41 ms) and only two out of six had persistent T wave alternans on ECG ( p  
ISSN:0147-8389
1540-8159
DOI:10.1111/pace.14827