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The aim was to determine the utility of electrophysiology (EP) study in children and young adults to risk stratify for sudden cardiac death (SCD). A retrospective cohort study was performed to evaluate the usefulness of EP study in predicting a composite endpoint of SCD or appropriate implantable cardioverter-defibrillator (ICD) discharge in subjects with and without congenital heart disease (CHD). Over the 14-year study period (1995–2009), 169 subjects underwent EP study at a median age of 15.1 years (IQR 12.1–18.7) and median follow-up of 4.7 years (IQR 1.8–8.8). Eighty-five subjects had CHD and 84 subjects did not have CHD. In the With CHD group, 25 of 85 subjects (31%) had positive EP study defined as inducible hemodynamically significant ventricular tachycardia or ventricular fibrillation. An ICD was placed in 18 subjects, with five subjects having subsequent appropriate ICD discharge. Of the 60 out of 85 subjects (69%) with negative EP study, there were two ICDs implanted and no appropriate ICD discharges. Subjects with positive EP study had significantly improved freedom from composite outcome of SCD or ICD discharge (log rank p1⁄4 0.002). In the Without CHD group, 25 of 84 subjects (30%) had a positive EP study, and ICDs were implanted in 11 subjects, with one subsequent appropriate ICD discharge. Of the 59 of 84 (70%) subjects with negative EP study, eight had ICDs implanted with two appropriate ICD discharges. There was no significant difference in freedom from composite outcome in subjects with positive and negative EP study. There was no SCD in either group. In our cohort of patients, EP study had limited utility for risk stratification. However, a negative EP study in CHD subjects may suggest a low SCD risk.

Diagnostic Electrophysiology Study Has Limited Value in Risk Stratification of Children and Young Adults with and without Congenital Heart Disease