Secondary Logo

Institutional members access full text with Ovid®

Implantable cardioverter-defibrillators in congenital heart disease

Mondésert, Blandine; Khairy, Paul

Current Opinion in Cardiology: January 2014 - Volume 29 - Issue 1 - p 45–52
doi: 10.1097/HCO.0000000000000019
ARRHYTHMIAS: Edited by David Birnie

Purpose of review 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.

Recent finding 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.

Summary 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:

© 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins