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

• Published in: European journal of pediatrics Vol. 168; no. 7; pp. 771 - 777
• Main Authors: Baruteau, Alban-Elouen, Baruteau, Julien, Joomye, Ryad, Martins, Raphael, Treguer, Frédéric, Baruteau, Remi, Daubert, Jean-Claude, Mabo, Philippe, Roussey, Michel
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01.07.2009; Springer; Springer Nature B.V; Springer Verlag [1975-....]
• Subjects: Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Babies; Bioengineering; Biological and medical sciences; Cardiac arrhythmia; Cardiac dysrhythmias; Cardiology and cardiovascular system; Cardiology. Vascular system; Computer Science; Congenital diseases; Electrocardiography; Emergency and intensive care: neonates and children. Prematurity. Sudden death; Engineering Sciences; Epidemiology; Family; General aspects; Genetic Predisposition to Disease; Heart; Human health and pathology; Humans; Infant; Infant, Newborn; Intensive care medicine; Life Sciences; Long QT Syndrome; Long QT Syndrome - complications; Long QT Syndrome - congenital; Long QT Syndrome - diagnosis; Long QT Syndrome - physiopathology; Medical sciences; Medicine; Medicine & Public Health; Mutation; Pediatrics; Postmortem Changes; Review; Risk Factors; SIDS; Signal and Image processing; Sudden Infant Death; Sudden Infant Death - etiology; Sudden infant death syndrome; Ventricular Fibrillation; Ventricular Fibrillation - complications; Ventricular Fibrillation - etiology; Ventricular Fibrillation - physiopathology; France; Familial screening; Sudden infant death syndrome (SIDS); Primary electrical disease; Cot death; Long-QT syndrome (LQTS); Pediatrics; Arrhythmia; Cardiovascular disease; Infant; Electrodiagnosis; Heart block; Heart disease; Primary; Electrocardiography; Sudden infant death syndrome; Human; Congenital; Family study; Sudden death; Mortality; Prolonged QT interval; Medical screening; Conduction disorder; Congenital disease; Sudden unexplained death
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-009-0951-y

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%. 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.