European Heart Journal Advance Access published online on May 22, 2009
European Heart Journal, doi:10.1093/eurheartj/ehp179
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Ventricular arrhythmias and sudden death in adults after a Mustard operation for transposition of the great arteries
1 Departement of Cardiology, Grown-up Congenital Heart Disease Program, University of Bern, Inselspital, Bern, Switzerland
2 Division of Cardiology, McMaster University Medical Center, Hamilton, ON, Canada
3 Division of Cardiology, Toronto Congenital Cardiac Center for Adults, University of Toronto, University Health Network, Toronto General Hospital, 585 University Avenue, 5 N-521 North Wing, Toronto, ON, Canada M5G 2N2
4 Division of Cardiology, University of Western Ontario, London, ON, Canada
5 Department of Medicine, Philadelphia Adult Congenital Heart Center, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA
Received 15 October 2008; revised 16 February 2009; accepted 7 April 2009 * Corresponding author. Tel: +1 416 340 3146, Fax: +1 416 340 5014, Email: candice.silversides{at}uhn.on.ca
Aims: To examine the prevalence of sustained ventricular tachycardia (VT) and sudden death (SD) in adults with atrial repair of transposition of the great arteries (TGA) and to determine associated risk factors.
Methods and results: In a single-centre review, we studied the outcome of 149 adults (mean age 28 ± 7 years) who had undergone a Mustard operation for TGA. During a mean follow-up of 9 ± 6 years, sustained VT and/or SD occurred in 9% (13/149) of the cohort. Sustained VT/SD was more likely to occur in patients with associated anatomic lesions [hazard ratio (HR) 4.9, 95% CI 1.5–16.0], with NYHA class 
III (HR 9.8, 95% CI 3.0–31.6) and with an impaired subaortic right ventricular (RV) ejection fraction (EF) (HR 2.2, 95% CI 1.2–4.0 per 10% decrease in EF). There was an inverse correlation between the RV-EF and both age and QRS duration. Patients with a QRS duration 140 ms were at highest risk of sustained VT/SD (HR 13.6, 95% CI 2.9–63.4). Atrial tachyarrhythmia was detected in 66 (44%) patients, but was not a statistically significant predictor of sustained VT/SD in our adult population (HR 2.7, 95% CI 0.6–13.0).
Conclusion: Sustained VT/SD in adults after a Mustard operation for TGA are more common than previously described. Age, systemic ventricular function, and QRS duration are interrelated and are associated with VT/SD. A QRS duration 140 ms helps to identify the high risk patient.
Key Words: Transposition of great vessels • Tachyarrhythmias • Survival