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Comparative Study
. 2012 Feb 7;59(6):607-15.
doi: 10.1016/j.jacc.2011.10.878.

Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy: a report from the Pediatric Cardiomyopathy Registry

Elfriede Pahl  1 , Lynn A Sleeper, Charles E Canter, Daphne T Hsu, Minmin Lu, Steven A Webber, Steven D Colan, Paul F Kantor, Melanie D Everitt, Jeffrey A Towbin, John L Jefferies, Beth D Kaufman, James D Wilkinson, Steven E Lipshultz; Pediatric Cardiomyopathy Registry Investigators
Affiliations
Comparative Study

Incidence of and risk factors for sudden cardiac death in children with dilated cardiomyopathy: a report from the Pediatric Cardiomyopathy Registry

Elfriede Pahl et al. J Am Coll Cardiol. .

Abstract

Objectives: The purpose of this study was to establish the incidence of and risk factors for sudden cardiac death (SCD) in pediatric dilated cardiomyopathy (DCM).

Background: The incidence of SCD in children with DCM is unknown. The ability to predict patients at high risk of SCD will help to define who may benefit most from implantable cardioverter-defibrillators.

Methods: The cohort was 1,803 children in the PCMR (Pediatric Cardiomyopathy Registry) with a diagnosis of DCM from 1990 to 2009. Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using Classification and Regression Tree methodology.

Results: The 5-year incidence rates were 29% for heart transplantation, 12.1% non-SCD, 4.0% death from unknown cause, and 2.4% for SCD. Of 280 deaths, 35 were SCD, and the cause was unknown for 56. The 5-year incidence rate for SCD incorporating a subset of the unknown deaths is 3%. Patients receiving antiarrhythmic medication were at higher risk of SCD (hazard ratio: 3.0, 95% confidence interval: 1.1 to 8.3, p = 0.025). A risk stratification model based on most recent echocardiographic values had 86% sensitivity and 57% specificity. Thirty of 35 SCDs occurred in patients who met all these criteria: left ventricular (LV) end-systolic dimension z-score >2.6, age at diagnosis younger than 14.3 years, and the LV posterior wall thickness to end-diastolic dimension ratio <0.14. Sex, ethnicity, cause of DCM, and family history were not associated with SCD.

Conclusions: The 5-year incidence rate of SCD in children with DCM is 3%. A risk stratification rule (86% sensitivity) included age at diagnosis younger than 14.3 years, LV dilation, and LV posterior wall thinning. Patients who consistently meet these criteria should be considered for implantable cardioverter-defibrillator placement.

Trial registration: ClinicalTrials.gov NCT00005391.

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Figures

Figure 1
Figure 1
Competing risks analysis for sudden cardiac death, non-sudden cardiac death, unknown cause of death and cardiac transplantation among 1803 children with dilated cardiomyopathy (DCM) listed in the Pediatric Cardiomyopathy Registry. The 3, 5, and 10-year cumulative incidence rates (95% CI) of sudden cardiac death are estimated to be 2.0% (1.4%-2.8%), 2.4% (1.7%-3.4%), and 2.7% (1.8%-3.9%); non-sudden cardiac death 10.8% (9.3%-12.4%), 12.1% (10.4%-13.9%) and 14.9% (12.6%-17.3%); heart transplant 27% (25%-29%), 29% (27% - 32%), and 31% (28% - 34%). The rate of death from unknown cause (95% CI) was 3.4% (2.6%-4.5%), 4.0% (3.0%-5.2%), and 5.2% (3.6%-7.3%).
Figure 2
Figure 2
Classification and Regression Tree for Sudden Cardiac Death (SCD): Predictors from Time of Cardiomyopathy Diagnosis (Total N=1747, 35 sudden cardiac deaths). Red boxes denote high-risk patient groups. Bold black boxes denote lower-risk patient subgroups. Sensitivity=57%, specificity=78%. False positive rate=95%, false negative rate=1.1%.
Figure 3
Figure 3
Classification and Regression Tree for Sudden Cardiac Death (SCD): Predictors from Time of Last Follow-up (Total N=1747, 35 sudden cardiac deaths). Red box denotes the high-risk patient group. Bold black boxes denote lower-risk patient subgroups. Sensitivity=86%, Specificity=57%. False positive rate=96%, false negative rate=0.6%.
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References

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