Published online February 1, 2007
PEDIATRICS
Vol. 119
No. 2
February 2007, pp.
407-408
(doi:10.1542/peds.2006-3083)
Preventing Pediatric Sudden Cardiac Death
Steven M. Yabek, MD
Pediatric Cardiology Associates
Albuquerque, NM 87106
To the Editor.—
Campbell and Berger1 should be congratulated for their recent commentary in Pediatrics, which raised much-needed awareness about sudden cardiac death (SCD) in pediatric patients. Although SCD is not a common occurrence, the authors correctly alluded to the devastating effects that such an unexpected event has on families and the community. They proposed decreasing SCD by encouraging the routine use of a questionnaire that stresses the patient and family history and referring to a pediatric cardiologist those patients deemed to be at high risk on the basis of positive responses. I propose that current evidence suggests the need for a routine diagnostic screening test, specifically a 12-lead electrocardiogram (ECG), in addition to a carefully obtained history and physical examination to significantly reduce the incidence of SCD.
The authors stated that 
40% of pediatric SCD victims may be identified prospectively by obtaining a thorough personal and family history. Even if accurate, I do not believe that 40% would, by anyone's criteria, define an appropriate screening process. More importantly, I believe that the number is probably overstated. In a retrospective study of SCD among 134 trained athletes (mean age: 17 years), Maron et al2 reported that only 3% might have been suspected on the basis of the standard preparticipation history and physical examination. More recently, in a study of 241 cases of SCD among young people,3 only 10% of the 70 patients who died from a lethal arrhythmia had a positive family history, although most deaths were probably related to an autosomal dominant genetic channelopathy.
In the United States, current screening practices rely exclusively on the history and physical examination and are based on recommendations that are a decade old. During that time, there has been no demonstrable change in the incidence of SCD among young people. In distinction, since 1982, all competitive athletes (12–35 years old) in Italy have been required to obtain a 12-lead ECG in addition to the usual preparticipation history and physical examination.4 As a result of this change, the annual incidence of SCD in young athletes in the Veneto region of Italy decreased by 89%, from 3.6 in 100 000 to 0.4 in 100 000 per year.4 In the unscreened nonathletic population, the SCD incidence did not change. Moreover, SCD from cardiomyopathies decreased 90% (from 1.5 to 0.15 in 100 000 per year), with the greatest decreases occurring in patients with hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy.
A large proportion of anatomic and electrical diseases that underlie SCD are detectable by ECG. I recognize the considerable logistic and financial limitations to widespread ECG screening. However, we universally screen for numerous endocrine and metabolic disorders that occur far less frequently than hypertrophic cardiomyopathy (1:500) which, in the United States, still accounts for more SCD in young people than any other diagnosis. Information acquired over the past decade has taught us that many of the genetically determined entities that predispose to SCD are clinically silent and are infrequently associated with a positive family history.
REFERENCES
- Campbell RM, Berger S. Preventing pediatric sudden cardiac death: where do we start?
Pediatrics. 2006;118
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–803[Free Full Text]
- Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, Mueller FO. Sudden death in young competitive athletes: clinical, demographic, and pathological profiles.
JAMA. 1996;276
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- Puranik R, Chow CK, Duflou JA, Kilborn MJ, McGuire MA. Sudden death in the young.
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- Corrado D, Basso C, Pavei A, Michieli P, Schiavon M, Thiene G. Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program.
JAMA. 2006;296
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PEDIATRICS (ISSN 1098-4275). ©2007 by the American Academy of Pediatrics
