Clinical Profile of Pediatric Patients with Long QT Syndrome Masquerading as Seizures

Abstract

LQTS can cause seizures due to prolonged ventricular arrhythmias in high risk subgroup. Children, who present with LQTS and seizures, generally have a precipitating event causing seizures, and they respond well to drug therapy.

Nicorandil, a Nitric Oxide Donor and ATP-Sensitive Potassium Channel Opener, Protects Against Dystrophin-Deficient Cardiomyopathy

“…The safety and efficacy of nicorandil have not yet been established in children, although there are case reports of using nicorandil in children for the treatment of congenital long QT syndrome, an inherited arrhythmia that carries a risk of sudden death. 58 Therefore, before initiating a clinical trial, it would be prudent to further understand the chronic effects of nicorandil on both cardiac and skeletal muscle structure and function in an animal model of Duchenne muscular dystrophy.…”

Pediatrik disritmiler

“…Uzun QT Sendromları, EKG'de QT intervalinde uzama ile karakterize gecikmiş ventrikül repolarizasyonu olan hastalık grubudur [2,[9][10][11]. QT uzamasının sebebi herediter veya edinsel olabilir.…”

“…Jervell-Lange-Nielsen sendromu, otozomal resesif geçişli bir bozukluk olup, QT mesafesinde uzama, doğuştan sağırlık, senkop ve ani ölüm ile karakterizedir [1,3,10].…”

“…Romano Ward sendromu ise otozomal dominant geçiş ile karakterize bir uzun QT sendromudur. Sağırlık yoktur, ancak senkop ve ani ölüm riski mevcuttur [3,5,10]. Jervell-Lange-Nielsen sendromundan daha sık görülür.…”