Electrocardiogram screening for disorders that cause sudden cardiac death in asymptomatic children: a meta-analysis

Area of Science:

• Pediatric Cardiology
• Clinical Screening
• Diagnostic Test Performance

Background:

• Pediatric sudden cardiac death (SCD) affects 0.8–6.2 per 100,000 children annually.
• Screening for cardiac disorders in asymptomatic children is appealing but the performance of electrocardiograms (ECGs) is unknown.
• Key disorders associated with pediatric SCD detectable by ECG include hypertrophic cardiomyopathy (HCM), long QT syndrome (LQTS), and Wolff-Parkinson-White syndrome.

Purpose of the Study:

• To estimate the phenotypic prevalence of HCM, LQTS, and Wolff-Parkinson-White syndrome in asymptomatic children.
• To determine the sensitivity, specificity, and predictive value of ECG, alone or with echocardiogram (ECHO), for detecting these SCD-associated disorders.

Main Methods:

• Systematic literature review of studies on HCM, LQTS, and Wolff-Parkinson-White syndrome.
• Data extraction from 30 selected articles after screening 6954 abstracts.
• Calculation of phenotypic prevalences and receiver operating characteristic (ROC) curve analysis for ECG performance.

Main Results:

• Phenotypic prevalences per 100,000 children: HCM (45), LQTS (7), Wolff-Parkinson-White (136).
• ECG demonstrated high areas under the ROC curve for detecting HCM (0.91) and LQTS (0.92).
• ECG showed high negative predictive value for HCM or LQTS, but positive predictive value varied with sensitivity, specificity, and prevalence.

Conclusions:

• The findings provide an evidence base for evaluating pediatric screening strategies for SCD-associated cardiac disorders.
• ECG is a sensitive screening test with high negative predictive value for mass screening.
• The utility of ECG screening is limited by variable positive predictive value and false-positive rates, necessitating careful interpretation.