JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

VOL. 65, NO. 21, 2015

ª 2015 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION

ISSN 0735-1097/$36.00

PUBLISHED BY ELSEVIER INC.

http://dx.doi.org/10.1016/j.jacc.2015.04.004

EDITORIAL COMMENT

Sudden Cardiac Death in Pediatric Cardiomyopathy The Importance of Well-Designed Population-Based Studies* Joseph W. Rossano, MD, Matthew J. O’Connor, MD

P

ediatric cardiomyopathies, although rare in

understanding of the incidence and risk factors for

the general population, are associated with

sudden cardiac death (SCD) in this population. The

significant morbidity, mortality, and cost (1).

SEE PAGE 2302

Much of our knowledge of the epidemiology and outcomes of these diseases can be traced to the 2 large,

study team investigated the incidence of SCD with 4

prospective, population-based studies that began in

of the major cardiomyopathy subtypes encountered

late 1980s and early 1990s: the Pediatric Cardiomyop-

in children: dilated cardiomyopathy (DCM), hyper-

athy Registry from North America and the National

trophic cardiomyopathy (HCM), restrictive cardio-

Australian Childhood Cardiomyopathy Study from

myopathy (RCM), and left ventricular noncompaction

Australia (2–5). The National Australian Childhood

(LVNC). From this analysis, risk factors for SCD were

Cardiomyopathy Study was initiated in 1987 and

determined. With a median follow-up of just over 11

enrolled 301 children diagnosed with cardiomyopathy

years, the cumulative incidence of SCD for the entire

at