Key result
Anti-arrhythmic medications linked to ~200% higher SCD risk in pediatric DCM.
Why the study?
The incidence of sudden cardiac death in children with dilated cardiomyopathy was unknown, and predicting high-risk patients is needed to define who may benefit most from implantable cardioverter-defibrillators.
What are the incidence rates and risk factors for sudden cardiac death in children with dilated cardiomyopathy?
Cohort (n=1,803)
What are the incidence rates and risk factors for sudden cardiac death in children with dilated cardiomyopathy?
Hazard Ratio: 3 (95% CI 1.1–8.3)
p-value: p=0.025
The 5-year incidence of sudden cardiac death in pediatric dilated cardiomyopathy is approximately 3%, and patients with specific echocardiographic criteria (LV dilation, posterior wall thinning) and age <14.3 years are at highest risk and may benefit from ICD placement.
Captured external expert commentary on this paper, strongest first. Original sources are linked where available.
“Prevention of Sudden Death for Patients With Cardiomyopathies: Another Step Forward”
“The PCMR collaborators have been instrumental in providing a rich description of childhood DCM, allowing a better understanding of etiology-specific outcomes, recovery of left ventricular function, incidence and risk factors for sudden cardiac death, and death while waiting for or after heart transplantation.”
Supports SCD risk stratification in pediatric DCM; leaves open prospective validation before guiding ICD decisions.
Objectives To establish the incidence of, and risk factors for, SCD in pediatric DCM. Background The incidence of SCD in children with DCM is unknown. The ability to predict patients at high risk for SCD will help define who may benefit most from ICDs. Methods The cohort was 1803 children in the PCMR diagnosed with DCM from 1990-2009. Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using CART methodology. Results Five-year incidence rates were 29% for heart transplant, 12.1% non-sudden cardiac death (non-SCD), 4.0% death from unknown cause, and 2.4% for SCD. Of 280 deaths, 35 were SCD and cause was unknown for 56. The 5-year rate for SCD incorporating a subset of the unknown deaths is 3%. Patients receiving anti-arrhythmic medication were at higher risk of SCD (hazard ratio 3.0, 95% CI 1.1-8.3, 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 of these criteria: LV end-systolic dimension z score > 2.6, age at diagnosis <14.3 years, and ratio of LVPWT:EDD <0.14. Sex, ethnicity, cause of DCM, and family history were not associated with SCD. Conclusions The 5-year incidence of SCD in children with DCM is 3%. A risk stratification rule (86% sensitivity) included diagnosis age < 14.3 years, LV dilation, and LV posterior wall thinning. Patients who consistently meet these criteria should be considered for ICD placement.
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Pahl et al. (2012) conducted a cohort in Dilated Cardiomyopathy (n=1,803). Anti-arrhythmic medication vs. No anti-arrhythmic medication was evaluated on Sudden cardiac death (SCD) (HR 3.0, 95% CI 1.1-8.3, p=0.025). In children with dilated cardiomyopathy, the 5-year incidence of sudden cardiac death was 3%, and anti-arrhythmic medication use was associated with increased risk (HR 3.0; 95% CI 1.1-8.3; P=0.025).
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