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R A Ildarova1, M A Shkolnikova, S A Termosesov
1Clinical Institute of Pediatrics named after Academician Y. E. Veltishev; RNIMU after N.I. Pirogov. ildarova@pedklin.ru.
Insights
Implantable cardioverter-defibrillators (ICDs) effectively prevent sudden cardiac death in children with long-QT syndrome. This study assessed ICD therapy outcomes and optimized its use in pediatric patients.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Genetics
Background:
- Long-QT syndrome (LQTS) is a genetic disorder predisposing to life-threatening ventricular arrhythmias.
- Sudden cardiac death (SCD) is a significant risk in pediatric LQTS patients.
- Current management often involves antiarrhythmic drugs, but ICDs are considered for high-risk individuals.
Purpose of the Study:
- To evaluate the effectiveness and safety of implantable cardioverter-defibrillator (ICD) therapy in children with LQTS.
- To analyze the clinical course of LQTS before and after ICD implantation.
- To optimize indications for ICD therapy in pediatric LQTS.
Main Methods:
- A cohort of 48 children with LQTS who received ICDs was compared to 59 children treated with beta-blockers.
- Clinical and electrocardiographic data were collected at baseline and during follow-up.
- Mean age at ICD implantation was 11.8 years, with a mean follow-up of 5.2 years.
Main Results:
- Children receiving ICDs typically had prolonged QT intervals (>500 ms), recurrent syncope, and a history of sudden cardiac arrest.
- These patients often required high-dose beta-blockers for arrhythmia control.
- ICD implantation was associated with effective management of ventricular tachyarrhythmias.
Conclusions:
- ICD implantation is a safe and effective strategy for both primary and secondary prevention of SCD in pediatric LQTS.
- The study supports the use of ICDs in carefully selected high-risk children with LQTS.
- Optimization of ICD therapy indications can improve outcomes in this population.
Purpose:
to assess specificities of course of the long-QT syndrome in children before and after implantation of cardioverter-defibrillator (ICD), and optimization of indications to ICD-therapy.
Materials And Methods:
We included in this study 48 children with long-QT syndrome from 44 unrelated families (28 boys and 20 girls), who underwent ICD implantation at the mean age 11.8±3.8 years. Mean duration of follow-up after implantation was 5.2±2.8 years. Data from these children were compared with those from 59 children of comparable age and gender with long-QT syndrome from 46 unrelated families receiving antiarrhythmic therapy (β-adrenoblockers). We assessed clinical and electrocardiographic characteristics of the disease obtained at initial visit and their dynamics thereafter.
Results:
Children with long-QT syndrome and ICD were mainly probands with interval QT longer than 500 ms, recurrent syncope and often history of sudden cardiac arrest requiring high doses of β-adrenoblockers for control of ventricular tachyarrhythmias.
Conclusion:
ICD implantation is an effective and safe method both of primary and secondary prevention of sudden cardiac death in children with long-QT syndrome.