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Original Article

A Prospective, Population-Based Study of Sudden Cardiac Death in the Young

Richard D. Bagnall, Ph.D., Robert G. Weintraub, M.B., B.S., Jodie Ingles, Grad.Dip.Gen.Couns., Ph.D., M.P.H., Johan Duflou, M.B., Ch.B.., M.Med., Laura Yeates, B.Sc., Grad.Dip.Gen.Couns., Lien Lam, Ph.D., Andrew Davis, M.B., B.S., M.D., Tina Thompson, B.Nurs, Vanessa Connell, Dip.App Sci., Jennie Wallace, R.N., Charles Naylor, M.A., M.B., B.Chir., Jackie Crawford, R.N., Donald R. Love, Ph.D., Lavinia Hallam, B.Sc., M.B., B.Ch., Jodi White, M.B., B.S., Christopher Lawrence, M.B., B.S., Matthew Lynch, L.L.B., M.B., B.S., Natalie Morgan, R.N., Grad.Dip.Genetic.Couns., Paul James, Ph.D., M.D., Desirée du Sart, Ph.D., Rajesh Puranik, M.B., Ph.D., Neil Langlois, M.B., B.Chir., M.A., M.D., Jitendra Vohra, M.D., Ingrid Winship, M.B., Ch.B., M.D., John Atherton, M.B., B.S., Ph.D, Julie McGaughran, M.B., Ch.B., M.D., Jonathan R. Skinner, M.B., Ch.B.., M.D., and Christopher Semsarian, M.B., B.S., Ph.D., M.P.H.

N Engl J Med | June 22, 2016 |DOI: 10.1056/NEJMoa1602002

Sudden cardiac death in the young is a devastating event. We performed a prospective, population-based, clinical and genetic study of sudden cardiac death in the young.

We prospectively collected clinical, demographic and autopsy information on all sudden cardiac death cases aged 1-35 years in Australia and New Zealand from 2010 to 2012. In cases with no cause identified on conventional autopsy (sudden unexplained death), at least 59 cardiac genes were analysed.

Four hundred ninety sudden cardiac death cases were identified. The annual incidence was 1.3 per 100,000 persons aged 1-35 years. Seventy-two percent were male. The age group at highest risk for sudden cardiac death was 31-35 years (3.2 per 100,000) and for sudden unexplained death was 16-20 years (0.83 per 100,000). Coronary artery disease (24% of cases) and inherited cardiomyopathies (16%) were the most common explained causes of sudden cardiac death. Sudden unexplained death (40% of cases) was the predominant finding in all age sub-groups, except those 31-35 years where coronary artery disease was most common. Younger age and death at night were independently associated with sudden unexplained death compared to explained sudden cardiac death. A clinically relevant cardiac gene mutation was identified in 27% of cases of sudden unexplained death. A clinical diagnosis of an inherited cardiovascular disease was identified in 12% of families during follow-up.

Autopsy investigation combined with genetic testing substantially increased the likelihood of identifying a possible cause of death in young individuals with sudden cardiac death over autopsy investigation alone.

 Originally Appeared in The New England Journal of Medicine on June 22, 2016.

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