The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair. Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD. This was a retrospective, multicenter, case-controlled study. We identified 47 patients after Mustard's or Senning's operation who experienced an SD event (34 SD, 13 near-miss SD). Each patient was matched with two controls with the same operation, but without an SD event. Information on numerous variables before the event was obtained and compared with controls at the same time frame. Presence of symptoms of arrhythmia or heart failure at most recent follow-up and history of documented arrhythmia (atrial flutter [AFL]/atrial fibrillation [AF]) were found to increase the risk of SD. Electrocardiogram (ECG), chest X-ray, and Holter ECG findings were not predictive of SD. Neither medication nor pacing was found to be protective. Most SD events (81%) occurred during exercise. Ventricular tachycardia/ventricular fibrillation were the recorded rhythm during SD in 21 of 47 patients. Presence of symptoms and documented AFL/AF are the best predictors of SD in TGA patients. Patients with these findings should be further evaluated for risk of SD.,
Journal of the American College of Cardiology
Department of Pediatrics

Kammeraad, J., van Deurzen, C., Sreeram, N., Bink-Boelkens, M., Ottenkamp, J., Helbing, W., … Balaji, S. (2004). Predictors of sudden cardiac death after mustard or senning repair for transposition of the great arteries. Journal of the American College of Cardiology, 44(5), 1095–1102. doi:10.1016/j.jacc.2004.05.073