Sudden Cardiac Death in Children and Adolescents

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Citation
US Cardiology, 2006;3(2):1-4

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In children and young adults, sudden cardiac death (SCD) occurs in approximately 1-2 per 100,000 population.1,2 While the overwhelming majority of occurrences of SCD occur in the adult population and are due to ischemic heart disease, very few cases in a pediatric population are. Pediatric SCD is a relatively uncommon occurrence whose underlying etiology often remains elusive. A long list of relatively rare and varied conditions accounts for SCD in previously well children.2-5 The implications for recurrences and risk in other family members are far-reaching, and more poignant than in the case of adult ischemic heart disease.The consequences for the family are devastating and the lack of an explanation leaves the family searching and uncertain of how to proceed.

Diagnostic Approach

A detailed history is potentially life-saving when seeing the young survivor of resuscitated SCD or a family with a history of SCD.When possible, booking a longer appointment is advisable. The family often has a lot to discuss; extended family may attend; and there are usually a lot of unresolved psychosocial issues that arise. Since the number of 'near-missesÔÇÖ for each condition causing SCD is unknown, each is an opportunity to prevent SCD in the future. There are often warning clues available in the history. It is imperative to get a detailed history of the circumstances preceding the event. Although it may seem difficult to discuss the event with the family, most families are eager to do whatever it takes to uncover an etiology for SCD. Collapse or syncope during exertion is common to a variety of causes of SCD and not usually a distinguishing feature. Death during sleep is seen in some forms of long QT syndrome and Brugada syndrome. The patientÔÇÖs exercise tolerance cannot be taken at face value; a detailed understanding of what sort of exertional effort was required to 'keep upÔÇÖ is needed. Similarly, the family history must be explored in depth for cases of 'faintingÔÇÖ, 'heart attackÔÇÖ and 'seizuresÔÇÖ. One must know whether one is dealing with a positive family history and a heritable condition, or a sporadic case.

The physical examination is directed at uncovering signs of cardiovascular disease. The general appearance and growth parameters will provide clues about whether the SCD is due to an undiagnosed chronic condition, such as cardiomyopathy or underlying heart disease. The precordium may be active or the apex displaced in cases of cardiac enlargement.

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