A Case of Congenital Long OT Syndrome with Pseudo - Atrioventricular Block

ABSTRACT

The congenital long-QT syndrome (LQTS) is characterized by recurrent syncope, prolonged QT intervals, QT interval lability, polymorphic ventricular tachycardia, and sudden death. We report a case of congenital long QT syndrome in a 28-day-old male infant who presented with syncope, bradycardia with 2: 1 pseudo-atrioventricular block and a markedly prolonged QT inteval. One episode occured after crying and degenerated into ventricular fibrillation and terminated after cardioversion. A VVI type cardiac pacemaker was implanted. Subsequently, the infant's heart rate was over 110/min and 2: 1 AV block and any other arrhythmia were absent. The infant recovered from the accompanied pneumonia and sepsis and was discharged 47 days after adrnission. However, 13 days after discharge, the infant returned to our hospital ER with syncope. Ventricular fibrillation ceased after cardioversion. Despite medication with propranolol, ventricular tachycardia persisted. The infant expired the day after he was discharged against medical advice.