Current trends in implantable cardioverter-defibrillator therapy in children ― Results from the JCDTR database ―

Background: Implantable cardioverter defibrillators (ICDs) are being used with increasing frequency in children. Our aim was to examine the current trend of pediatric ICD implantation in Japan. Methods and Results: Data was extracted from the Japanese Cardiac Device Treatment Registry (JCDTR), a nation-wide registry started in 2006. All patients aged less than 18 years who had an ICD implantation registered between 2006 and 2016 were included in the analysis. A total of 201 patients were included, with a median age of 16 years (range 1–18). The underlying cardiac diagnosis was primary electrical disease (67%), cardiomyopathy (26%) and congenital heart disease (4%), with idiopathic ventricular fibrillation (29%) and long QT syndrome (21%) being the 2 most common diagnoses. Implantation indication was primary prevention in only 30 patients (15%). There were 27 patients (13%) aged ≤12 years, with a larger proportion of patients with cardiomyopathy (33%). The indication in younger children was secondary prevention in all cases. Younger children may be under-represented in our study given the nature of the database as it is a predominantly adult cardiology database. Conclusions: In the past decade, ICD implantation has been performed in approximately 20 children per year in Japan, mostly for secondary prevention. There was no increase in the trend nor a change in age distribution.