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CONGENITAL HEART DISEASE

Predictors of sudden cardiac death after mustard or senning repair for transposition of the great arteries

Janneke A.E. Kammeraad, MD^*, Carolien H.M. van Deurzen, MD^*, Narayanswami Sreeram, MD^*, Margreet Th.E. Bink-Boelkens, MD, Jaap Ottenkamp, MD, Willem A. Helbing, MD, Jan Lam, MD^||, Martha A. Sobotka-Plojhar, MD^¶, Otto Daniels, MD^# and Seshadri Balaji, MD^**,*

^* Department of Pediatric Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands
Department of Pediatric Cardiology, University Hospital Groningen, Groningen, the Netherlands
Department of Pediatric Cardiology, Center for Anomalies of the Heart Amsterdam/Leiden (CAHAL), Leiden, the Netherlands
Department of Pediatric Cardiology, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, the Netherlands
^|| Department of Pediatric Cardiology, CAHAL, Amsterdam, the Netherlands
^¶ Department of Pediatric Cardiology, CAHAL, Amsterdam, the Netherlands
^# Department of Pediatric Cardiology, University Hospital Nijmegen, Nijmegen, the Netherlands
^** Department of Pediatric Cardiology, Oregon Health Sciences University, Portland, Oregon, USA

Manuscript received December 22, 2003; revised manuscript received April 28, 2004, accepted May 18, 2004.

^* Reprint requests and correspondence: Dr. Seshadri Balaji, Department of Pediatric Cardiology, Oregon Health and Science University, 707 SW Gaines Road, Mailcode CDRC-P, Portland, Oregon 97239 (Email: balajis{at}ohsu.edu).

OBJECTIVES: 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.

BACKGROUND: Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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.

Abbreviations and Acronyms   AF = atrial fibrillation   AFL = atrial flutter   CT = cardiothoracic   ICD = implantable cardioverter-defibrillator   RV = right ventricle/ventricular   SD = sudden death   SND = sinus node disease   TGA = transposition of the great arteries   VF = ventricular fibrillation   VT = ventricular tachycardia

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