Role of congenital long-QT syndrome in unexplained sudden infant death: proposal for an electrocardiographic screening in relatives.

Introduction: Congenital long-QT syndrome (LQTS) is a sporadic or familial inherited arrhythmia. It can lead to sudden death by ventricular fibrillation which occurs at any age but particularly during infancy. Recent studies of postmortem molecular analysis in infants who died of unexplained sudden infant death syndrome (SIDS) showed abnormal mutations to LQTS in 10% to 12%.

[Long QT syndrome: an underestimated cause of sudden infant death].

Congenital long QT syndrome (LQTS) is an inherited arrhythmia that can be sporadic or familial. It predisposes to sudden cardiac death by ventricular fibrillation, which can occur at any age, particularly in neonates. Recent postmortem molecular screening surveys have shown that 10 to 12% of sudden infant death syndrome (SIDS) cases were potentially related to congenital long QT syndrome.