Primary Prevention Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease: Recommendations From Professional Societies

Identifying adults with congenital heart disease (CHD) at high risk for sudden death for primary-prevention implantable cardioverter defibrillators (ICDs) remains a major challenge. Inconsistencies among professional-society recommendations are a source of confusion. This article summarizes and compares recommendations from the Canadian Cardiovascular Society (CCS) (2009), Pediatric and Congenital Electrophysiology Society (PACES) and Heart Rhythm Society (HRS) (2014), European Society of Cardiology (ESC) sudden death guidelines (2015), European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and ESC Working Group on Grown-Up CHD (2018), and ESC guidelines on adult congenital heart disease (2020). Major areas over which there is consensus are presented and scenarios with continued clinical uncertainty, which offer important opportunities for future research are highlighted.