A prospective study of sudden cardiac death among children and young adults

Bagnall, R. D., Weintraub, R. G., Ingles, J., Duflou, J., Yeates, L., Lam, L., Davis, A. M., Thompson, T., Connell, V., Wallace, J., Naylor, C., Crawford, J., Love, D. R., Hallam, L., White, J., Lawrence, C., Lynch, M., Morgan, N., James, P., du Sart, D., Puranik, R., Langlois, N., Vohra, J., Winship, I., Atherton, J., McGaughran, J., Skinner, J. R. and Semsarian, C. (2016) A prospective study of sudden cardiac death among children and young adults. New England Journal of Medicine, 374 25: 2441-2452. doi:10.1056/NEJMoa1510687


Author Bagnall, R. D.
Weintraub, R. G.
Ingles, J.
Duflou, J.
Yeates, L.
Lam, L.
Davis, A. M.
Thompson, T.
Connell, V.
Wallace, J.
Naylor, C.
Crawford, J.
Love, D. R.
Hallam, L.
White, J.
Lawrence, C.
Lynch, M.
Morgan, N.
James, P.
du Sart, D.
Puranik, R.
Langlois, N.
Vohra, J.
Winship, I.
Atherton, J.
McGaughran, J.
Skinner, J. R.
Semsarian, C.
Title A prospective study of sudden cardiac death among children and young adults
Journal name New England Journal of Medicine   Check publisher's open access policy
ISSN 1533-4406
0028-4793
Publication date 2016-06-23
Year available 2016
Sub-type Article (original research)
DOI 10.1056/NEJMoa1510687
Open Access Status Not Open Access
Volume 374
Issue 25
Start page 2441
End page 2452
Total pages 12
Place of publication Waltham, United States
Publisher Massachussetts Medical Society
Collection year 2017
Language eng
Formatted abstract
BACKGROUND
Sudden cardiac death among children and young adults is a devastating event. We performed a prospective, population-based, clinical and genetic study of sudden cardiac death among children and young adults.

METHODS

We prospectively collected clinical, demographic, and autopsy information on all cases of sudden cardiac death among children and young adults 1 to 35 years of age in Australia and New Zealand from 2010 through 2012. In cases that had no cause identified after a comprehensive autopsy that included toxicologic and histologic studies (unexplained sudden cardiac death), at least 59 cardiac genes were analyzed for a clinically relevant cardiac gene mutation.

RESULTS
A total of 490 cases of sudden cardiac death were identified. The annual incidence was 1.3 cases per 100,000 persons 1 to 35 years of age; 72% of the cases involved boys or young men. Persons 31 to 35 years of age had the highest incidence of sudden cardiac death (3.2 cases per 100,000 persons per year), and persons 16 to 20 years of age had the highest incidence of unexplained sudden cardiac death (0.8 cases per 100,000 persons per year). The most common explained causes of sudden cardiac death were coronary artery disease (24% of cases) and inherited cardiomyopathies (16% of cases). Unexplained sudden cardiac death (40% of cases) was the predominant finding among persons in all age groups, except for those 31 to 35 years of age, for whom coronary artery disease was the most common finding. Younger age and death at night were independently associated with unexplained sudden cardiac death as compared with explained sudden cardiac death. A clinically relevant cardiac gene mutation was identified in 31 of 113 cases (27%) of unexplained sudden cardiac death in which genetic testing was performed. During follow-up, a clinical diagnosis of an inherited cardiovascular disease was identified in 13% of the families in which an unexplained sudden cardiac death occurred.

CONCLUSIONS
The addition of genetic testing to autopsy investigation substantially increased the identification of a possible cause of sudden cardiac death among children and young adults. (Funded by the National Health and Medical Research Council of Australia and others.)
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Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
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