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

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 <.01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD. Conclusion: Jervell Lange‐Nielsen syndrome is a subtype of LQTS with high‐risk 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. [ABSTRACT FROM AUTHOR]