Dr. Leslie Cooper has shared this Abstract that was recently published in the New England Journal of Medicine June 23, 2016. See the highlights below and read the whole abstract below.
As many of you have so sadly realized, sudden cardiac death among children and young adults is a devastating event. A study on 490 cases of sudden cardiac death among children and young adults ages 1 to 35 years-of-age was conducted in Australia and New Zealand from 2010 through 2012.
Unexplained sudden cardiac death 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.
In this study, men and boys had a higher incidence than did women and girls, and persons 16-20 years of age had the highest incidence of unexplained sudden cardiac death.
Myocarditis was the cause of death in 7% of the cases. 40% of the cases had no definitive cause.
Unexplained sudden cardiac death is often attributed to cardiac arrhythmia which is undetectable in a conventional autopsy. Autopsy investigation combined with genetic testing and family screening was associated with a substantially higher likelihood of identifying a possible cause of death among children and young adults who had a sudden cardiac death than was autopsy investigation alone.
Thus it is key that we continue to educate the public and medical communities on myocarditis.
ABSTRACT
A Prospective Study of Sudden Cardiac Death among Children and Young Adults
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 his
-tologic 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 unex
-plained 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. Dur
-ing 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.)
N Engl J Med 2016;374:2441-52.
Authors: R.D. Bagnall, R.G. Weintraub, J. Ingles, J. Duflou, L. Yeates, L. Lam, A.M. Davis,
T. Thompson, V. Connell, J. Wallace, C. Naylor, J. Crawford, D.R. Love, L. Hallam,
J. White, C. Lawrence, M. Lynch, N. Morgan, P. James, D. duSart, R. Puranik,
N. Langlois, J. Vohra, I. Winship, J. Atherton, J. McGaughran, J.R. Skinner,
and C. Semsarian
