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Congenital heart disease
Screening for potentially fatal heart disease in children and teenagers
  1. Christopher Wren
  1. Correspondence to Dr Christopher Wren, Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK; christopher.wren{at}nuth.nhs.uk

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The sudden death of a child or teenager is a rare event with a profound impact on parents, family, friends and schoolmates. It often generates much publicity and instils fear in the parents of other healthy children. If the postmortem examination reveals a previously unsuspected cardiac abnormality, this raises the question of whether it could have been detected and death could have been prevented. If no cause is found at autopsy, and a cardiac arrhythmia is presumed to be responsible, there is no satisfactory resolution for the parents. Screening programmes for “young athletes” have been proposed in Europe and the USA based on the consensus views of experts and informed mainly by data from sudden deaths up to the age of 35 years.w1 This review will examine the feasibility of detection of potentially fatal latent heart defects and the possibility of screening in children and teenagers.

Incidence of sudden death

Specific data on unexpected sudden death in children and teenagers are not easy to find. Corrado et al reported a rate of 1.0/105 in Italians up to the age of 35 years.1 The risk increased with age in this population so the proportion younger than 20 years was probably less than half. A report from an International Olympic Committee working group found that 40% of such deaths occurred under the age of 18 years.2 Maron reported a sudden death risk of 0.46/105 person years in high school athletes.3 Van Camp reported a rate of 0.4/105 in high school and college athletes, being 0.75/105 in males and 0.13/105 in females.w2 A study of cardiac arrest in schools estimated an annual incidence of 0.18/105 person-years among students.w3

It is reasonable to conclude that the overall average risk of sudden cardiac death in apparently normal …

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