Abstract
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. 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±3.8years. Mean duration of follow-up after implantation was 5.2±2.8 years. Data from these children were compared with those from 59 children ofcomparable 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. 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. ICD implantation is an effective and safe method both of primary and secondary prevention of sudden cardiac death inchildren with long-QT syndrome.
Highlights
We included in this study 48 children with long-QT syndrome
electrocardiographic characteristics of the disease obtained at initial visit
who's at the risk for sudden cardiac death
Summary
Purpose: to assess specificities of the long-QT syndrome in children before and after implantation of cardioverter-de fibrillator (ICD), and optimization of indications to ICD-therapy. В дальнейшем ИКД были широко внедрены в стандарты лечения пациентов из группы высокого риска с СУИQT, а устройства стали имплантировать не только больным, перенесшим опасные для жизни осложнения, но и пациентам без симптомов с доказанным риском, например, случаи ВСС в семье или синдрома LQT3 [5, 17, 18]. В группу сравнения были отобраны 59 детей с наследственным СУИQT без ИКД из 46 неродственных семей, сопоставимые по возрасту и полу с пациентами основной группы, получающие ААТ β-адреноблокаторами (34 мальчика и 25 девочек, средний возраст на момент первого визита в клинику 8,9±4,0 года). У всех больных оценивали данные анамнеза (наличие и частота синкопальных эпизодов до начала ААТ, наличие эпизодов ВОК, особенности течения заболевания на фоне ААТ); семейный анамнез (наличие СУИQT и случаев ВСС в семье у родственников I–II степени родства в возрасте до 40 лет).
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