State of Cardiac Implantable Electronic Devices in Congenital Heart Disease: A 2019 Update

Abstract

The prevalence of adult congenital heart disease (CHD) is directly attributable to the success of pediatric cardiology and congenital cardiac surgery in diagnosing and managing cardiac conditions in childhood. The observed success with this population has resulted in the application of electrophysiological techniques and therapies to an expanding population of patients, bypassing previous limitations secondary to size, underlying knowledge base, or both. Cardiac implantable electronic devices (CIEDs) are no exception and the last decade has seen a remarkable expansion into the CHD population. The 2014 Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS) expert consensus statement on the recognition and management of arrhythmias in adult CHD 1 and the 2018 American Heart Association/American College of Cardiology guidelines for the management of adults with CHD 2 underscore that the care of these patients continues to become formalized. The year 2019 saw the release of several studies examining the impact of CIEDs in CHD with a focus placed on subcutaneous implantable cardioverter defibrillators (S-ICDs), epicardial devices, cardiac resynchronization therapy (CRT) systems, and leadless pacing. Subcutaneous implantable cardioverter defibrillatorsThe release of the S-ICD was heralded as a result of its utility in patients with limited vascular access and contraindications to the implantation of transvenous systems. This, the lack of intravascular components, and the concomitant need for lead extraction made the S-ICD particularly favorable in the CHD population, where patients are typically younger and frequently present with atypical venous return. This year saw the release of several larger series describing the acute and midterm outcomes of S-ICD implantation and served to enhance our understanding of this device type in this challenging group of patients.At the 2019 HRS Scientific Sessions, a collaborative presentation organized together with the PACES described 116 pediatric and adult patients who had undergone S-ICD implantation, including 37 who had CHD. 3 Among the entire cohort, the investigators found that 97.9% of patients experienced a successful first-shock cardioversion during implant testing and 92.5% demonstrated the delivery of appropriate therapy during a follow-up period of 33 months ± 29 months. Complications were comparable to those reported in other studies of CIEDs in this population, with 18.9% of patients experiencing any of a variety of selected complications. Notably, infections requiring device removal were low (1.7%) and no cases of endocarditis were 3940