European Journal of Cardiovascular Medicine

Congenital long QT syndrome is an inherited cardiac disorder characterized by prolonged ventricular depolarization and risk of sudden cardiac death. A 40 year old female patient, resident of Murshidabad, West Bengal, presented to the ER with recurrent episodes of dizziness and 3 episodes of syncope in the last 3 months. Bradycardia can occur in all forms of congenital LQT syndromes. Loss of repolarizing IKs in LQT1 leads to profound increase in cycle length of SAN cells, thus generating sinus bradycardia. Also, many KCNQ1 mutations are associated with sinus bradycardia. Early diagnosis with implantation of a dual chamber ICD in long QT syndrome patients with bradycardia can definitely add years to life in these patients.