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Cardiac causes for syncope or sudden death in childhood
  1. CHRISTOPHER WREN, Consultant Paediatric Cardiologist
  1. Freeman Hospital, Newcastle upon Tyne NE7 7DN, UK

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    Sudden death of a child is every parent’s worst nightmare. It is a rare occurrence, although individual cases or causes may receive disproportionate attention in the press. Syncope, on the other hand, is a common symptom in childhood and is almost always benign. In a very few cases syncope may be a clue to the presence of an underlying cardiovascular problem and may predict a risk of sudden death. Retrospective assessment of victims of sudden death sometimes reveals a preceding history of syncope to suggest that earlier recognition of the problem might have led to a different outcome. This review considers the causes of sudden death, the causes of syncope, and which children with syncope need further investigation.

    Cardiac causes of sudden death in infancy

    Sudden death in infancy is usually either sudden infant death syndrome or caused by infection. A few neonatal or infant deaths are caused by congenital cardiovascular malformations, particularly duct dependent abnormalities or obstructive left heart malformations.1 Sustained tachycardia usually presents with heart failure but may cause collapse or even death. Atrioventricular re-entry tachycardia, the most common type of supraventricular tachycardia, maybe difficult to recognise in the neonate. The history is often non-specific and there are few physical signs. Ventricular arrhythmias in infancy are rare but some types are dangerous. Complete atrioventricular block is usually recognised in utero or soon after birth but may cause death if unrecognised or untreated.

    Sudden infant death syndrome implies absence of any identifiable cause of death. Extensive investigation has failed to demonstrate any cardiovascular contribution to sudden infant death syndrome2 and, despite recent reports, there is no convincing evidence that QT prolongation is implicated.3

    Sudden death in children with known heart disease

    In previous generations sudden cardiac death most often occurred in children with end stage pulmonary vascular disease associated with unoperated congenital heart disease or in children with unoperated aortic valve …

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