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

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.

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.

A retrospective cohort study of patients who underwent AR for ccTGA between 1993 and 2017 at a single center was conducted.

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.

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.