Abstract 18950: Invasive Electrophysiology Study has Higher Sensitivity and Specificity for Sudden Cardiac Death in Patients with Congenital Heart Disease than those with Structurally Normal Hearts

Abstract

Introduction: The use of electrophysiology study (EPS) in pediatric and congenital heart disease (CHD) patients to identify at risk subjects for sudden cardiac death (SCD) and implantable cardiac defibrillator (ICD) is often debated. Objectives: We assessed the hypothesis that EPS was more predictive for SCD or appropriate ICD discharge in patients with CHD compared to those with structurally normal hearts. Methods: A 15 year retrospective database review was performed for subjects undergoing EPS risk stratification for SCD. Positive EPS was defined as inducible sustained ventricular tachycardia or fibrillation by programmed stimulation. Data included demographics, EPS indications, outcome of EPS, ICD complications, and follow up SCD or ICD discharge. The primary endpoint was SCD or appropriate ICD discharge. Results: The study included169 subjects with mean age of 19.1 ± 7.8 years, mean follow up after EPS of 5.8 ± 4.3 years. Of these, 50 subjects (30%) had positive EPS, with ICDs subsequently placed in 28 (56%). There were 119 subjects (70%) with negative EPS, and ICDs were placed in 9 (7%). There were 85 subjects (50%) with CHD. There were no SCDs in either group. During follow-up, 10 of 37 subjects (24%) with ICDs had appropriate discharges (6 with CHD and 4 without CHD). Device related complications including recalls were seen in 21 of 37 patients (57%). The ICD shock rate, sensitivity and specificity of EPS are shown in the Table. Sub group analysis of right heart obstruction CHD lesions (n=44) showed similar results between these patients and all CHD patients (Table). Conclusions: The use of diagnostic EPS has a higher sensitivity and specificity for appropriate ICD discharge in pediatric patients with CHD compared to those with structurally normal hearts.