Sudden cardiac death is a leading cause of mortality in patients with congenital heart disease (CHD), such that implantable cardioverter-defibrillators (ICDs) are a critical component of care. Objectives of this review are to highlight recent advances regarding ICDs in CHD, with a focus on clinical indications, technical issues and solutions, and patient outcomes.
Evolving ICD indications in CHD are primarily derived from observational research or extrapolations from the general adult literature. Technical challenges to device implantation include obstructed vasculature or conduits, intracardiac shunts with their attendant risk for systemic thromboemboli, and lack of venous access to the heart. In selected patients, tailored epicardial systems may be considered that include subcutaneous, retrocardiac, and/or venous (e.g., azygous) coils. Alternatively, an entirely subcutaneous ICD may be a reasonable option in patients with no bradycardia or antitachycardia pacing indications. Long-term complications include inappropriate shocks, lead failure, reduction in quality of life, shock-related anxiety, and impaired sexual function.
Although ICDs undeniably save lives, challenges to applying this technology to patients with CHD include the paucity of evidence-based data to guide patient selection, technical challenges related to venous access, patient size, anatomic complexities, and a high rate of complications.
Adult Congenital Heart Center and Electrophysiology Service, Montreal Heart Institute, Université de Montreal, Montreal, Canada
Correspondence to Dr Paul Khairy, Adult Congenital Heart Center, Montreal Heart Institute, 5000 Belanger St E., Montreal, Quebec H1T 1C8, Canada. Tel: +1 514 376 3330 ext. 3800; fax: +1 514 593 2581; e-mail: email@example.com