Role of congenital long-QT syndrome in unexplained sudden infant death: proposal for an electrocardiographic screening in relatives.
BACKGROUND
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%. Current methods of etiologic investigation of sudden infant death syndrome do not allow the diagnosis of LQTS. A targeted anamnesis together with systematic electrocardiograms of first- and second-degree relatives could be an efficient LQTS diagnostic tool. Therefore, we propose to include them in screening procedures for SIDS etiologies.


CONCLUSIONS
LQTS accounts for a significant number of unexplained SIDS. We suggest adding a systematic familial electrocardiographic screening to the current etiologic investigations in order to track congenital LQTS in relatives.
Baruteau
Alban-Elouen
AE
CHU Rennes, Service de Cardiologie et Maladies Vasculaires, Rennes, France. alban.baruteau@wanadoo.fr
Baruteau
Julien
J
Joomye
Ryad
R
Martins
Raphael
R
Treguer
Frédéric
F
Baruteau
Remi
R
Daubert
Jean-Claude
JC
Mabo
Philippe
P
Roussey
Michel
M
D004562:Electrocardiography; D005190:Family; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000150:complications* / Q000151:congenital / Q000175:diagnosis* / Q000503:physiopathology; D011180:Postmortem Changes; D012307:Risk Factors; D013398:Sudden Infant Death / Q000209:etiology*; D014693:Ventricular Fibrillation / Q000150:complications* / Q000209:etiology / Q000503:physiopathology