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Catheter Ablation of Atrial Fibrillation in Transposition of
the Great Arteries Treated With Mustard Atrial Baffle
David S. Frankel, MD; Maully J. Shah, MBBS; Peter F. Aziz, MD; Mathew D. Hutchinson, MD
T
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he survival of patients with D-transposition of the great
arteries (D-TGA) has dramatically improved with corrective surgical procedures, including the atrial and arterial
switch operations. In the current era, the majority of the adult
patients typically have undergone atrial switch operations,
either a Senning or a Mustard procedure. Late complications
include baffle obstruction, systemic ventricular failure, systemic atrioventricular valve regurgitation, and rhythm disturbances. The most common atrial arrhythmia in this population is intra-atrial reentrant tachycardia (IART), which has
been associated with development of heart failure and
death.1– 4 Proarrhythmic factors include atrial enlargement
from tricuspid regurgitation and systemic right ventricular
failure, as well as suture lines. As this population ages,
chronic hemodynamic stress may result in atrial fibrillation as
well. We present the first report of catheter ablation for
treatment of atrial fibrillation in a patient with D-TGA treated
with prior Mustard repair.
vertically and then carried down along the pulmonary venous
pathway between the pulmonary veins to the base of the left
atrial appendage, extending through the narrowest portion of
the pathway. A large Gore-Tex patch was secured to the
pulmonary venous pathway. His systemic right ventricle was
moderately impaired at that time, with moderate tricuspid
regurgitation. Over the following 4 years, he had recurrent,
highly symptomatic atrial fibrillation requiring several DC
cardioversions despite treatment with dofetilide and then
amiodarone. After much discussion, catheter ablation was
recommended.
A CT scan (Figure 1D) was obtained before the ablation
both to facilitate procedural planning and to integrate with the
electroanatomic mapping system (NavX, St Jude Medical, St
Paul, MN). Intracardiac echocardiography (Acunav, Siemens,
Mountain View, CA) was used to guide catheter positioning
and monitor pulmonary venous flows (Figure 1C). The
patient was placed under general anesthesia, using a propofol
infusion, and was intubated for the procedure. A transbaffle
puncture was performed, using fluoroscopy and contrast
angiography to access the pulmonary venous atrium (Figure
1A and 1B). The circular mapping catheter (Lasso, Biosense
Webster, Diamond Bar, CA) was positioned in the left
common pulmonary vein and the ablation catheter in the right
inferior pulmonary vein (Figure 2B and 2C). Atrial fibrillation triggers were elicited with isoproterenol (3–30 ␮g/min)
and with atrial burst pacing. During infusion of isoproterenol,
isolated premature atrial depolarizations were recorded from
the right inferior pulmonary vein (Figure 2A, asterisk) and
atrial tachycardia was recorded from the left common pulmonary vein, which did not initiate atrial fibrillation (arrows).
Circumferential antral pulmonary vein isolation was performed; the right veins were targeted independently (Figure
3A). Ablation was performed with an open irrigated catheter
(EZ Steer Thermocool, Biosense Webster, Diamond Bar,
CA). Both entrance block (Figure 3B) and exit block (Figure
3C) were demonstrated in all 3 pulmonary vein ostia. After
ablation, isoproterenol was administered again, this time
without any triggers identified; dissociated firing within the
Case Report
A 41-year-old man with D-TGA status post Mustard repair
presented with longstanding, recurrent atrial arrhythmias. He
originally underwent balloon atrial septostomy 2 days after
birth and a subsequent atrial switch procedure using the
Mustard technique at 2 years of age.5 He did well until age
32, at which time he had recurrent symptomatic IART. EP
study was performed. Cavotricuspid isthmus (CTI)dependent flutter was induced and confirmed by entrainment
mapping. Lines of ablation were drawn across the systemic
venous baffle portion of the “medial” CTI and the pulmonary
venous “lateral” CTI using a retrograde, aortic approach.
Flutter terminated with ablation and could not be reinduced.
Bidirectional block was demonstrated across the CTI. He
remained arrhythmia-free for 2 years, then presented again at
age 34 with exertional intolerance and fatigue due to partial
obstruction of the pulmonary venous baffle. He underwent
patch augmentation of the stenotic pulmonary venous pathway and suture closure of a leak at the superior systemic
venous baffle. A right (systemic) atrial incision was made
Received December 14, 2011; accepted January 24, 2012.
From the Cardiovascular Division, Electrophysiology Section of the Hospital of the University of Pennsylvania (D.S.F., M.D.H.), and Children’s
Hospital of Philadelphia (M.J.S., P.F.A.), Philadelphia, PA.
Correspondence to David S. Frankel, MD, Cardiovascular Division, Electrophysiology Section, Hospital of the University of Pennsylvania, 9 Founders
Pavilion, 3400 Spruce St, Philadelphia PA 19104. E-mail [email protected]
(Circ Arrhythm Electrophysiol. 2012;5:e41-e43.)
© 2012 American Heart Association, Inc.
Circ Arrhythm Electrophysiol is available at http://circep.ahajournals.org
e41
DOI: 10.1161/CIRCEP.111.969857
e42
Circ Arrhythm Electrophysiol
April 2012
Figure 1. D-transposition of the great arteries with
Mustard atrial switch anatomy. A, Contrast angiography opacifies the inferior vena cava baffle, the
systemic venous atrium, and the superior vena
cava baffle. B, Transbaffle puncture is performed
with the second sheath now in the pulmonary
venous atrium. Contrast opacifies the right superior pulmonary vein as well as the pulmonary
venous atrium. C, The intracardiac echocardiography catheter, placed in the systemic venous
atrium, is used to guide positioning of the circular
mapping catheter (L) at the ostium of the left common pulmonary vein. D, Contrast enhanced CT
scan demonstrates the pulmonary venous atrium
and pulmonary veins in gray, and the systemic
venous atrium, inferior vena cava baffle, and superior vena cava baffle in purple. SVC indicates
superior vena cava; IVC, inferior vena cava; LCPV,
left common pulmonary vein; RIPV, right inferior
pulmonary vein; RSPV, right superior pulmonary
vein; and SVA, systemic venous atrium.
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pulmonary vein ostia was seen. Bidirectional block was
confirmed across the CTI, from the prior ablation. Aggressive
atrial burst pacing and programmed stimulation failed to
induce sustained arrhythmias.
There were no complications from the ablation procedure.
Dofetilide was restarted, in accordance with our practice of
treating patients with an antiarrhythmic drug for 6 weeks
following atrial fibrillation ablation. The patient was discharged on the second postoperative day. Over the following
6 months, he had 2 episodes of IART, with cycle length 290
ms, superiorly directed p wave, and 2:1 AV conduction. Both
episodes were symptomatic and required DC cardioversion.
He has had no recurrences of atrial fibrillation.
Discussion
To our knowledge, this is the first report of catheter ablation
for treatment of atrial fibrillation in a patient with D-TGA
status post Mustard atrial switch. Although survival of
patients with D-TGA has dramatically improved with corrective surgical procedures, atrial arrhythmias are a common late
sequela, approaching 25% and increasing with time.6 Although the most common incident arrhythmia is IART, atrial
fibrillation may occur as well. Electrophysiology studies have
confirmed abnormalities of sinus node function as well as
atrial conduction and refractoriness.7
Our patient had several risk factors for atrial arrhythmias,
including complex atrial reoperation, impaired systemic right
ventricular hemodynamics, and tricuspid regurgitation. Atrial
arrhythmias in patients with systemic right ventricular dysfunction are often highly symptomatic and poorly tolerated.
Although antiarrhythmic drugs are a reasonable first line of
treatment for atrial fibrillation, efficacy is often inadequate
and proarrhythmia may occur.8 Therefore, in patients with
D-TGA treated with Mustard atrial baffle, with recurrent
Figure 2. Pulmonary vein triggers. A, During isoproterenol infusion (30 ␮g/min), a premature depolarization is recorded from the right inferior pulmonary vein (3rd beat, asterisk) followed by atrial
tachycardia from the left common pulmonary vein
(4th through 9th beats, arrows). B, Right anterior
oblique projection with duodecapolar catheter
from the right internal jugular vein, extending
through the superior vena cava (SVC) baffle and
the systemic venous atrium into the inferior vena
cava (IVC) baffle; decapolar catheter positioned
within the systemic venous atrial appendage; circular mapping catheter positioned in left common
pulmonary vein; and ablation catheter positioned
in right inferior pulmonary vein. C, Left anterior
oblique projection of same catheter positions.
LCPV indicates left common pulmonary vein;
RIPV, right inferior pulmonary vein.
Frankel et al
AF Ablation in TGA s/p Mustard Atrial Baffle
e43
Figure 3. Catheter ablation. A, Circumferential
antral ablation was performed around the left and
right pulmonary veins. The left common pulmonary
vein was ablated as a single ostium. The right
superior and inferior pulmonary veins were ablated
as separate ostia. B, During ablation around the
left common pulmonary vein, entrance block is
achieved, with disappearance of pulmonary vein
potentials between the 2nd and 3rd beats (arrows).
C, Exit block is demonstrated by failure to capture
the atrium with pacing (10 mA, 2 ms) from contiguous lasso poles around the circumference of
each vein ostium. SVC indicates superior vena
cava; IVC, inferior vena cava; LCPV, left common
pulmonary vein; RIPV, right inferior pulmonary
vein; and RSPV, right superior pulmonary vein.
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atrial fibrillation despite antiarrhythmic drugs, catheter ablation is a viable treatment option. We were able to demonstrate
pulmonary vein triggers for atrial fibrillation, as is nearly
uniformly the case in patients without congenital heart
disease.9 We also showed that pulmonary vein isolation is
technically feasible using a transbaffle puncture approach to
access the pulmonary venous atrium. Further, by isolating the
pulmonary veins, atrial tachycardia could not be induced with
the same dose of isoproterenol that induced atrial tachycardia
before ablation. Last, our case highlights the limitations of
arrhythmia inducibility in the electrophysiology laboratory,
perhaps exacerbated by the use of general anesthesia. Best
catheter ablation results are likely to be achieved with
collaboration between adult and pediatric electrophysiologists and interventional cardiologists in this population with
unique challenges and risks.10
3.
4.
5.
6.
7.
8.
Acknowledgments
We thank Stephen Garafolo and Michael Calabro for their assistance
with electroanatomic mapping.
9.
Disclosures
None.
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KEY WORDS: atrial fibrillation 䡲 catheter ablation
isolation 䡲 transposition of great vessels 䡲 Mustard
䡲
pulmonary vein
Catheter Ablation of Atrial Fibrillation in Transposition of the Great Arteries Treated
With Mustard Atrial Baffle
David S. Frankel, Maully J. Shah, Peter F. Aziz and Mathew D. Hutchinson
Downloaded from http://circep.ahajournals.org/ by guest on May 2, 2017
Circ Arrhythm Electrophysiol. 2012;5:e41-e43
doi: 10.1161/CIRCEP.111.969857
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