The Sudden Death in the Young
Registry
Screening for Sudden Cardiac Death
in the Young
Report from a National Heart, Lung, and
Blood Institute Working Group
Jonathan R. Kaltman, MD; Paul D. Thompson, MD; John Lantos, MD; Charles I. Berul, MD; Jeffrey
Botkin, MD, MPH; Joshua T. Cohen, PhD; Nancy R. Cook, ScD; Domenico Corrado, MD, PhD;
Jonathan Drezner, MD; Kevin D. Frick, PhD; Stuart Goldman, MD; Mark Hlatky, MD; Prince J.
Kannankeril, MD; Laurel Leslie, MD, MPH; Silvia Priori, MD, PhD; J. Philip Saul, MD; Carrie K.
Shapiro-Mendoza, PhD, MPH; David Siscovick, MD, MPH; Victoria L. Vetter, MD; Robin Boineau,
MD; Kristin M. Burns, MD; Richard A. Friedman, MD
Circulation, 2011; 123: 1911-1918
Recommendations
Epidemiology and etiology of Sudden Cardiac Death in the Young (SCDY)
Develop an SCDY registry to prospectively estimate the incidence of SCDY
Perform case-control studies using registry-defined cases to identify risk factors for SCDY
Epidemiology and Etiology of Sudden
Cardiac Death in the Young (SCDY)
Performance of the screening methodology in the target population
Perform pilot ECG screening studies to test the characteristics of the ECG in target populations
Perform comparative effectiveness studies to determine the incremental value of various screening
methodologies, including history and physical examination, ECG, echocardiogram, and genetic
testing
Develop a registry to prospectively
estimate the incidence of SCDY

Management of asymptomatic heart disease identified by ECG screening
Develop evidence-based management strategies for asymptomatic patients
Evaluate risk stratification, prevention, and therapeutic strategies
Use novel study designs and innovative recruitment strategies when studying low-prevalence diseases
Perform case-control studies using
registry-defined cases to identify risk
factors for SCDY
Impact 
of a screening program
Evaluate the impact of a screening program on individuals and families using quality-of-life studies and
patient-preference measurements
Use decision analysis to evaluate overall effectiveness of a screening program, as well as cost and
resource utilization
Other resources to facilitate research
Redefine the diagnostic criteria for the long-QT syndrome in the genetic era
Define ECG norms in the US population, taking into account demographic variability
Sudden Death in the Young Registry
Phase I:
 Develop a surveillance system to identify SDY cases
 Create a registry of clinical information about each case
 death certificates, medical records, death scene
investigations, and pathology reports
 Obtain a biospecimen from cases
Phase II:
 Make the registry data available to investigators in
scientific community
 Support mechanistic, genetic, and other studies which
use the registry to evaluate causes of and risk factors
for SDY
Investigator
Phase I
Investigator
Phase II
Sudden Death in the Young (SDY) Registry
Data Coordinating
Center
Biorepository
Investigator
Timeline
Spring
2014
Summer
2014
• State public • Awards made
health agencies
via
apply to CDC
cooperative
FOA to
agreements
participate
with the CDC
January
2015
July
2018
• Data
collection
begins
• Funding
cycle ends
Sudden Unexpected Death Initiative
Case Ascertainment
Sudden
Death
Initial
Investigation
SDY Registry
Medical
Examiner
Biorepository
Child Death
Review
Advanced
Review
The Role of the Electrophysiologist
Advanced
Review
• SDY Teams in funded states will convene local
experts in pediatric cardiology, neurology, and
forensic pathology
• Experts will:
– Review comprehensive case records to determine the
cause of death
– Apply the SDY Registry Case Definition to classify cases
The Role of the Electrophysiologist
Advanced
Review
• We hope you will participate in an SDY team in
your state!
• Contact your state public health agency or
Child Death Review Team to encourage them
to apply or to join their team.
http://www.childdeathreview.org/state.htm
Questions?
Kristin M. Burns, M.D.
NHLBI Project Scientist for the
Sudden Death in the Young Registry
301-594-6859
Kristin.Burns@nih.gov