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Advances in the prevention of sudden cardiac death in the young
1 Signal Transduction Group, Centenary Institute, Sydney,
Australia
* To whom correspondence should be addressed.
Sudden cardiac death (SCD) is a tragic and devastating complication of a number
of cardiovascular diseases. Although coronary artery disease accounts for a
majority of these deaths across all ages, many other aetiologies contribute to this
problem when it occurs in the young (age Key Words: sudden cardiac death, screening, genes, family, multidisciplinary
First published on January 14, 2009, doi:10.1177/1753944708100181 |
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35 years),
where coronary artery disease is far less common. Specifically, genetic heart
disorders are an important cause of SCD in the young. While pharmacological
therapies have made some impact on prevention of SCD, the introduction of
implantable cardioverter-defibrillator (ICD) therapy has been the single major
advance in the prevention of SCD in the young. In addition, the awareness that most
causes of SCD in the young are inherited, means family screening of relatives of
young SCD victims allows identification of previously unrecognised at-risk
individuals thereby enabling prevention of SCD in relatives. The role of genetic
testing, both in living affected individuals and in the setting of a
‘molecular autopsy’, is emerging as a key factor in
early diagnosis of an underlying cardiovascular genetic disorder. Understanding the
genetic basis of SCD, investigating the molecular mechanisms that lead from the gene
defect to the clinical phenotype, and elucidating the specific environmental
triggers for SCD, will most likely lead to further key improvements in the
prevention of SCD in the young.