Long-Term Electrophysiologic Comorbidities After Anatomically Repaired Congenitally Corrected Transposition of the Great Arteries

BACKGROUND: Anatomic repair (AR) of congenitally corrected transposition of the great arteries (ccTGA) restores the morphologic right and left ventricles to their natural positions (subpulmonary and subsystemic, respectively). AR consists of a combined atrial switch and either an arterial switch or a Rastelli operation and is associated with unknown atrial arrthymia risk. Arrhythmia burden is presumed high, based on significant atrial arrhythmia burden for d-transposition of the great arteries after atrial switch operation, but published arrhythmia data specifically for anatomically repaired ccTGA patients are limited. OBJECTIVES: This study sought to assess the incidence and burden of mid- and long-term atrial arrhythmia associated with AR and to determine which anatomic, preoperative, operative, and postoperative characteristics were associated with increased risk and burden of atrial arrhythmias. METHODS: A retrospective cohort study of patients who underwent AR for ccTGA between 1993 and 2017 at a single center was conducted. RESULTS: During a median follow-up of 10.6 years, 28% (24/85) of the cohort experienced at least one significant atrial tachyarrhythmia (AT) at a median onset time of 9.0 years after AR. The need for atrioventricular valve reintervention increased long-term risk of AT (HR: 4.4; 95% CI: 1.4-13.8; P = 0.01). The majority of patients who experienced AT had a low disease burden. CONCLUSIONS: Patients who underwent AR are at significant risk for developing AT, even within a short timeframe after the index surgery, with increasing incidence over time. The need for atrioventricular valve reintervention after AR increases the risk of AT.