Abstract
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.
Highlights
Transposition of the great arteries (TGA) represents about 5% to 7% of all congenital heart disease, and is charac-terized by ventriculo-arterial discordance, wherein the pulmonary arteries originate from the left ventricle and the aorta arises from the right ventricle.[1,2] Based upon the atrioventricular concordance or discordance, the defect is further classified as dextro-transposition of the great arteries (d-TGA) or levo-transposition of the great arteries (l-TGA), respectively.[3]Children born with d-TGA are generally cyanotic and often require an operative intervention for survival.[2]
We examined the clinical experiences of a single, large referral center, in an effort to further understand the arrhythmic substrates of l-TGA and atrially corrected d-TGA, including the indications for device implantation and subsequent clinical course and outcomes
We identified and confirmed 71 patients with TGA, including 38 patients with d-TGA and 33 patients with l-TGA, who received an implantable cardiac device between January 1996 and June 2014
Summary
Transposition of the great arteries (TGA) represents about 5% to 7% of all congenital heart disease, and is charac-terized by ventriculo-arterial discordance, wherein the pulmonary arteries originate from the left ventricle and the aorta arises from the right ventricle.[1,2] Based upon the atrioventricular concordance or discordance, the defect is further classified as dextro-transposition of the great arteries (d-TGA) or levo-transposition of the great arteries (l-TGA), respectively.[3]Children born with d-TGA are generally cyanotic and often require an operative intervention for survival.[2]. ICD Implantation in Patients with Transposition of the Great Arteries patients with l-TGA, in the absence of associated lesions, may remain asymptomatic and progress into early and even later adulthood without the abnormality being recognized.[4]. Owing to the evolution of surgery in d-TGA and the progression into adulthood of patients with unidentified l-TGA, cardiologists encounter an increasing number of adults with TGA.[4,5,6] The most common problems affecting these patients long term include arrhythmias, heart failure, and sudden cardiac death (SCD).[3,7,8] Because of these complications, a large number of TGA patients eventually require pacemakers or implantable cardioverter-defibrillators (ICDs). While use of ICDs for secondary prevention is generally accepted, implantation for primary prevention remains a topic of great scrutiny
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