Implantation of Cardioverter-Defibrillator in Children With Long-QT Syndrome: Assessment of Indications, Efficacy, and Safety Based on 10-Year Experience

Abstract

Purpose: to assess specificities of course of the long­QT syndrome in children before and after implantation of cardioverter­defibrillator (ICD), and optimization of indications to ICD­therapy.Materials and methods. We included in this study 48 children with long­QT 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 follow­up 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 long­QT syndrome from 46 unrelated families receiving antiarrhythmic therapy (β­adrenoblockers). We assessed clinical and electrocardiographic characteristics of the disease obtained at initial visit and their dynamics thereafter.Results. Children with long­QT 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 β­adrenoblockers 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 long­QT syndrome.

A rare indication of permanent pacemaker implantation in children: congenital long QT syndrome

“…Because of the occasional malign arrhythmias, medical therapies (beta blockers and other antiarrhythmics) and implantable cardiac defibrillators (ICDs) are the most commonly used treatment modalities. 3,4 Sinus bradycardia accompanying congenital LQTS is a known condition. 5 It is suggested that various types of LQTS gene mutations are also expressed in the human sinoatrial node (SAN), but the current data on the main mechanisms of SAN dysfunction due to LQTS is limited.…”