Use of Implantable Cardioverter-Defibrillators in Congenital Heart Disease and Pediatric Patients: Results from the German National Registry for Congenital Heart Defects

Background: Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts. Methods and results: Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75-46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to 2015, only 0.2% of the CHD patients in the national register received an ICD. Indication for ICD implantation was secondary prevention in 84 patients (78%) and primary prevention in 24 patients (22%). 23 patients (21%) of the ICD patients received appropriate ICD therapy. 7 patients (6%) received an inappropriate ICD therapy. In 23 patients (21%) device complications were documented with a high number of lead fractures and insulation defects (n = 14, 13%). Conclusion: The current study investigates the clinical uptake and use of ICD therapy based on a large national registry for CHD patients. Despite a steady increase in the number of implanted devices, ICD uptake remains relatively low, particularly for primary prevention. The data suggests a potential reluctance in utilization of device therapy in this patient cohort for primary prevention. Selecting patients in whom benefits outweigh the risks associated with lifelong ICD therapy remains challenging.