Pacemaker and Defibrillator Implantation in Patients with Transposition of the Great Arteries

Transposition of the great arteries (TGA) is represented in 5% to 7% of patients with congenital heart disease. These patients face a significant burden of arrhythmia and sudden cardiac death throughout their lives, and many eventually undergo pacemaker or cardiac-defibrillator implantation. Outcomes data following device implantation in this population, however, are limited. From an electrophysiologic database at a large, tertiary care medical center, we identified 63 TGA patients (34 with dextro (d)-TGA and 29 with levo (l)-TGA) with systemic right ventricles receiving an implantable cardiac device from 1996 to 2014. Medical records were reviewed for demographic, echocardiography and device interrogation data. Overall, l-TGA patients were older than d-TGA patients when they underwent initial device implantation (35.6 ± 18.2 versus 17.3 ± 10.6 years, p<0.001), and had more concomitant cardiac defects (55% versus 12%, p<0.001). Survival following initial device implantation was similar between l-TGA and d-TGA (72% versus 74%, p = 1.00), despite the baseline difference in age. Twenty-four patients underwent implantable cardioverter-defibrillator (ICD) implantation: 18 for primary intervention (11 l-TGA and seven d-TGA), and six for secondary prevention (four l-TGA and two d-TGA). Sixty-seven percent of patients in the secondary prevention group had appropriate shocks, compared with 0% of primary prevention patients. Patients with ICD discharge were more likely to have concomitant heart defects (100% versus 30%, p = 0.011). Despite being significantly younger, d-TGA patients had similar survival rates following device implant to l-TGA patients. Patients with TGA and sustained ventricular arrhythmias are at high risk for subsequent events, and typically benefit from ICD implantation. The role of prophylactic ICD implantation in this population, however, remains uncertain.