Jervell and Lange-Nielsen syndrome. Family case (clinical observation)

Abstract

A clinical family case of Jervell and Lange-Nielsen syndrome in 2 children is presented. Long QT syndrome is one of the leading causes of sudden cardiac death in children. Jervell and Lange-Nielsen syndrome is one of the most severe and rare types of long QT syndrome. Symptoms of the disease appear in infancy; they are characterized by a lengthening of QT interval on ECG, syncope as a result of life-threatening ventricular tachycardia and ventricular fibrillation, combined with congenital bilateral hearing loss.