title: Sudden unexplained death in infants and children: the role of undiagnosed inherited cardiac conditions.
abstract: Sudden unexplained death in childhood is a traumatic event for both the immediate family and medical professionals. This is termed sudden unexplained or arrhythmic death syndrome (SUDS/SADS) for children over 1 year of age while sudden unexplained death in infancy or sudden infant death syndrome (SUDI/SIDS) refers to unexplained deaths in the first year of life. There is increasing evidence for the role of undiagnosed inherited cardiac conditions, particularly channelopathies, as the cause of these deaths. This has far-reaching implications for the family regarding the potential risk to other family members and future pregnancies, providing a challenge not only in the counselling but also in the structured assessment and management of immediate relatives. This review will discuss the cardiac risk involved in sudden unexplained deaths of infants and children, the role of molecular autopsy, family cardiological screening, current management strategies, and future directions in this area.
authors.affiliation: Cardiovascular Sciences Research Centre, Division of Clinical Sciences, St George's University of London, Cranmer Terrace, London SW17 0RE, UK.
authors.forename: Leonie C H
authors.initials: LC
authors.lastname: Wong
authors.affiliation: Cardiovascular Sciences Research Centre, Division of Clinical Sciences, St George's University of London, Cranmer Terrace, London SW17 0RE, UK ebehr@sgul.ac.uk.
authors.forename: Elijah R
authors.initials: ER
authors.lastname: Behr
mesh_terms: D001145:Arrhythmias, Cardiac / Q000151:congenital* / Q000175:diagnosis / Q000401:mortality*; D053840:Brugada Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality*; D002423:Cause of Death; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D005820:Genetic Testing / Q000379:methods*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D057089:Pathology, Molecular / Q000379:methods*; D018570:Risk Assessment / Q000379:methods*
keywords: Inherited cardiac conditions; Molecular autopsy; Sudden arrhythmic death syndrome; Sudden infant death syndrome