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Sodium channel blocker in therapy of type 3 long Q–T syndrome
https://doi.org/10.21508/1027-4065-2025-70-2-30-37
Abstract
Long QT syndrome caused by a mutation in the SCN5A gene is the third most common molecular genetic type of the syndrome. Cardiac events in long QT syndrome type 3 are associated with a high risk of death, and beta-blocker therapy is not effective enough. Given the impaired inactivation of the cardiomyocyte sodium channel due to variants in the SCN5A gene, sodium channel blockers have been proposed as gene-specific therapy.
The aim of this study was to evaluate the efficacy and safety of therapy with the sodium channel blocker lappaconitine hydrobromide in a cohort of children with type 3 long QT syndrome.
Materials and methods. The study included 16 patients from 11 unrelated families who received combination therapy with a betablocker and lappaconitine hydrobromide for 48±37 months.
Results. The main indication for the use of lappaconitine hydrobromide was a pronounced prolongation of the QT interval (in 81.25% of cases). The duration of the QTc interval significantly decreased after the administration of lappaconitine hydrobromide both according to the standard ECG (from 507±20.7 to 451±26.0 ms; p=0.000003) and during Holter monitoring (mean QTc from 536±16.2 to 489±24.9 ms; p=0.0002). No spontaneous arrhythmic events were noted during combined antiarrhythmic therapy.
Conclusion. The sodium channel blocker lappaconitine hydrobromide in patients with type 3 long QT syndrome affects such a risk factor for sudden cardiac death as the QTc interval by significantly reducing its duration. The drug can be recommended for use in addition to beta-blocker therapy for QTc ≥ 500 ms on a standard ECG or mean QTc during Holter monitoring in patients with type 3 of the syndrome.
About the Authors
R. A. Ildarova
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Moscow
V. V. Bereznitskaya
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Moscow
M. A. Shkolnikova
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Moscow
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Review
For citations:
Ildarova R.A., Bereznitskaya V.V., Shkolnikova M.A. Sodium channel blocker in therapy of type 3 long Q–T syndrome. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2025;70(2):30-37. (In Russ.) https://doi.org/10.21508/1027-4065-2025-70-2-30-37
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