Published Online
on September 12, 2008

A more recent version of this article appeared on October 1, 2008

This Article Full Text (PDF) All Versions of this Article: 1/4/250    most recent CIRCEP.108.776120v1 Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Khairy, P. Articles by Cecchin, F. Search for Related Content PubMed PubMed Citation Articles by Khairy, P. Articles by Cecchin, F. Related Collections Ablation/ICD/surgery Pediatric and congenital heart disease, including cardiovascular surgery Related Article

Original Article

Sudden Death and Defibrillators in Transposition of the Great Arteries with Intra-atrial Baffles: A Multicenter Study

Paul Khairy^1,5; Louise Harris²; Michael J. Landzberg³; Susan M. Fernandes³; Amanda Barlow²; Lise-Andrée Mercier; Sangeetha Viswanathan⁴; Philippe Chetaille⁴; Elaine Gordon²; Annie Dore and Frank Cecchin³

¹ Adult Congenital Heart Center, Montreal Heart Institute, Montreal; Children's Hospital, Boston;
² Canadian Adult Congenital Heart (CACH) Network;
³ Children's Hospital Boston;
⁴ Leeds General Infirmary

⁵ E-mail: paul.khairy{at}cardio.chboston.org

Background—Transposition of the great arteries (D-TGA) with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter-defibrillators (ICDs).

Methods and Results—We conducted a multicenter cohort study in patients with D-TGA to determine actuarial rates of ICD shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients, age 28.0±7.6 years (89.2% male), were enrolled from 7 sites. ICDs were implanted for primary prevention in 23 (62.1%) and secondary prevention in 14 (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio 18.0, P=0.0341) and lack of beta-blockers (hazard ratio 16.7, P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison to 37.5% of non-inducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio 0.91 per kg, P=0.0168). Additionally, 14 (37.8%) patients experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator-related, and 12 (32.4%) late lead-related.

Conclusion—In patients with D-TGA, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias, beta-blockers appear protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.

Key Words: death, sudden (if surviving, use heart arrest) • defibrillation • heart defects, congenital • tachyarrhythmias • transposition of great vessels

Related Article

Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra-atrial Baffles: A Multicenter Study

Paul Khairy, Louise Harris, Michael J. Landzberg, Susan M. Fernandes, Amanda Barlow, Lise-Andrée Mercier, Sangeetha Viswanathan, Philippe Chetaille, Elaine Gordon, Annie Dore, and Frank Cecchin
Circ Arrhythm Electrophysiol 2008 1: 250-257. [Abstract] [Full Text] [PDF]