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

By Alban-Elouen Baruteau, Julien Baruteau, Ryad Joomye, Raphael Martins, Frédéric Treguer, Remi Baruteau, Jean-Claude Daubert, Philippe Mabo and Michel Roussey

Abstract

International audienceINTRODUCTION: 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. CONCLUSION: 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

Topics: Sudden infant death syndrome (SIDS), Cot death, Long-QT syndrome (LQTS), Primary electrical disease, Familial screening, MESH : Electrocardiography, MESH : Family, MESH : Ventricular Fibrillation, MESH : Genetic Predisposition to Disease, MESH : Humans, MESH : Infant, MESH : Infant, Newborn, MESH : Long QT Syndrome, MESH : Postmortem Changes, MESH : Risk Factors, MESH : Sudden Infant Death, [ SDV.IB ] Life Sciences [q-bio]/Bioengineering, [ SPI.SIGNAL ] Engineering Sciences [physics]/Signal and Image processing, [ INFO.INFO-TS ] Computer Science [cs]/Signal and Image Processing, [ SDV.MHEP.PED ] Life Sciences [q-bio]/Human health and pathology/Pediatrics, [ SDV.MHEP.CSC ] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system
Publisher: Springer Verlag
Year: 2009
DOI identifier: 10.1007/s00431-009-0951-y
OAI identifier: oai:HAL:hal-00911293v1
Provided by: Hal-Diderot
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