Cardiovascular genomics and sudden cardiac death in the young.

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Abstract

BACKGROUND:

Sudden cardiac death (SCD) in the young is a rare but tragic consequence of a number of genetic cardiovascular disorders. The care of survivors of cardiac arrest and families affected by SCD seeks to prevent further SCD events through family screening.

OBJECTIVE:

The aim of this article is to review the genetic basis of SCD in the young and outline the clinical aspects of caring for families affected by SCD.

DISCUSSION:

Inherited cardiomyopathies and primary arrhythmia syndromes are important causes of SCD in young people. Over the past 30 years, there has been an explosion of knowledge regarding the underlying genetic mechanisms of these disorders and dramatic advances in genetic testing technologies. Family screening with thorough multidisciplinary clinical assessment and genetic testing allows for the initiation of preventive strategies in high-risk relatives and ultimately a reduction in SCD events.