Implantable cardiac defibrillator among adults with transposition of the great arteries and atrial switch operation: case series and review of literature

Background: The experience with the implantable cardiac defibrillator (ICD) in patients with transposition of the great arteries (TGA) and history of atrial switch surgery remains limited.

Methods: Retrospective evaluation aiming to assess characteristics and outcomes of consecutive TGA patients with history of atrial switch surgery implanted with an ICD between January 2005 and June 2012 in four French centers.

Results: Of the 12 patients (median 34 years [28, 40]; 67% male), 4 patients (33%) were implanted for secondary prevention after symptomatic documented sustained ventricular tachycardia or sudden cardiac arrest. ICDs were implanted for primary prevention in 8 patients (67%), including cardiac resynchronization in 3 patients; severe systemic ventricle dysfunction was present in all cases (median ejection fraction 27% [20, 40]). Overall, one patient died during the ICD implantation secondary to refractory cardiac arrest after defibrillation testing. Over a median follow-up of 19 months [10, 106], 6 patients out of 11 (54%) experienced worsening of congestive heart failure, including 5 who were eventually transplanted. Overall, 3 patients (27%) experienced significant ICD-related complications, whereas only one patient (primary prevention indication) developed appropriate ICD therapy (successful anti-tachycardia pacing without shock). Half of the patients presented with at least one episode of sustained (≥ 5 min) atrial arrhythmia during follow-up.

Conclusions: Our findings underline the key role of progressive heart failure in dictating outcomes among TGA patients with prior atrial switch repair. Our results also underline the need of better risk-stratification for sudden cardiac death in those patients.