Complete transposition of the great arteries (D-TGA) accounts for 5% to 7% of congenital
heart defects. Although the arterial switch procedure has now replaced atrial redirection
as the surgical procedure of choice, most adults today with D-TGA have had Mustard
or Senning baffles. These surgeries involve extensive atrial reconstruction and predispose
to sinus node dysfunction and atrial tachyarrhythmias.
1
,
2
By 20 years after surgery, the prevalence of atrial tachyarrhythmias is approximately
25%, continues to increase with time, and is similar among patients with Mustard or
Senning baffles.
1
,
2
In our experience, intra-atrial reentrant tachycardia (IART) is the most common arrhythmia
substrate, followed by nonautomatic focal atrial tachycardia (NAFAT) and atrioventricular
(AV) nodal reentrant tachycardia.
3
,
4
Accessory-pathway-mediated tachyarrhythmias and atrial fibrillation occur less frequently.
5
Table 1 summarizes the arrhythmias encountered in D-TGA.
Table 1Arrhythmias in transposition of the great arteries with Mustard or Senning baffles
| Type of arrhythmia | Relative prevalence | Comments |
|---|---|---|
| Bradyarrhythmia | ||
| Sinus node dysfunction | ***** | More so in Mustard than Senning baffles |
| AV block | ** | Predominantly if associated VSD or tricuspid valve surgery |
| Tachyarrhythmia | ||
| IART | **** | Most common atrial tachyarrhythmia |
| Rotating around tricuspid valve | **** | Circuit typically includes portion in pulmonary venous atrium |
| Other | ** | Circuit involving superior baffle lines is next most common |
| Focal atrial tachycardia | *** | May coexist with IART |
| Nonautomatic | *** | Predominantly around suture lines |
| Automatic | ** | Typically junctional in nature |
| AV nodal reentrant tachycardia | ** | Slow pathway most often in pulmonary venous atrium |
| Atrial fibrillation | * | Some forms of IART may resemble atrial fibrillation |
| Accessory-pathway-mediated tachycardia | * | Rare, unlike congenitally corrected transposition |
| Ventricular tachycardia/fibrillation | ** | May be primary or secondary to atrial tachyarrhythmias |
Asterisks represent a relative ordinal scale ranging from uncommon (*) to highly prevalent
(*****).
AV = atrioventricular; IART = intra-atrial reentrant tachycardia; VSD = ventricular
septal defect.
Keywords
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References
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- Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures.Cardiol Young. 2004; 14: 284-292
- The electrophysiology of atrioventricular nodal reentry tachycardia following the Mustard or Senning procedure and its radiofrequency ablation.Cardiol Young. 2005; 15: 611-616
- Mapping and radiofrequency ablation of intraatrial reentrant tachycardia after the Senning or Mustard procedure for transposition of the great arteries.Am J Cardiol. 1996; 77: 985-991
- Wolff-Parkinson-white syndrome in d-transposition of the great arteries and mustard baffle.Heart Rhythm. 2006; 3: 853-856
- A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects.J Am Coll Cardiol. 1998; 32: 245-251
- Comparison of long-term outcomes of atrial repair of simple transposition with implications for a late arterial switch strategy.Circulation. 1999; 100: II176-II181
- Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.Circ Arrhythmia Electrophysiol. 2008; 1: 250-257
- Arrhythmia and survival in patients >18 years of age after the Mustard procedure for complete transposition of the great arteries.Am J Cardiol. 1999; 83: 1080-1084
- Radiofrequency catheter ablation of supraventricular tachycardia substrates after Mustard and Senning operations for d-transposition of the great arteries.J Am Coll Cardiol. 2000; 35: 428-441
Article info
Publication history
Published online: November 28, 2008
Accepted:
November 15,
2008
Received:
October 29,
2008
Footnotes
This work was supported in part by the Canada Research Chair in Electrophysiology and Adult Congenital Heart Disease (Dr. Khairy). Dr. Khairy has received honoraria from St Jude Medical.
Identification
Copyright
© 2009 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.
