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Case Reports
Acta Cardiol Sin 2008;24:51-5
Transcatheter Closure of Atrial Septal Defect
in Ebstein’s Anomaly Using the
Amplatzer Septal Occluder
Ming-Chien Wu, Chih-Yuan Fang, Mien-Cheng Chen and Morgan Fu
Ebstein’s anomaly is frequently associated with atrial septal defect (ASD). This interatrial communication also
contributes to systemic desaturation, systemic embolization, pulmonary hypertension, and right-side heart failure.
We describe a case of a 25-year-old lady who had central cyanosis and exercise intolerance due to Ebstein’s anomaly
with ASD. These manifestations markedly improved following closure of the ASD using an Amplatzer septal
occluder. In the literature, transcatheter closure device for ASD is safe and effective, however, this has rarely been
reported regarding Ebstein’s anomaly.
Key Words:
Ebstein’s anomaly · Atrial septal defect · Amplatzer septal occluder
INTRODUCTION
overloads. Closure of interatrial communication might
prevent, improve or resolve these conditions. In the current era, transcatheter closure can be performed safely
and successfully.7 Percutaneous closure of Ebstein’s anomaly-associated ASD is rarely reported.8 We report our
experience of transcatheter closure of ASD using Amplatzer septal occluder in a case with Ebstein’s anomaly.
In patients with Ebstein’s anomaly, a portion of the
right ventricle (RV) is atrialized, and the functional RV
is small. In addition, most patients have some interatrial
communication [atrial septal defect (ASD) or patent foramen ovale (PFO)] through which right-to-left shunting
may occur.1 The clinical presentation varies with age.
Rhythm abnormality is the most common presentation in
adolescents and adults with Ebstein’s anomaly,2 and is
the major cause of significant morbidity and sudden
death.3-6
In infants and children, right-to-left atrial shunting
can result in cyanosis. The interatrial communication
also contributes to late right ventricular failure, arrhythmia, and pulmonary hypertension due to right ventricle
CASE REPORT
A 25-year-old lady had uncertain diagnosed heart
disease since the age of six. She had exercise intolerance, easy fatigue and cyanosis of nail bed since childhood. However, she hadn’t experienced orthopnea, paroxysmal nocturnal dyspnea, or leg edema before. She
suffered from sudden onset of dyspnea and palpitation
on the morning of admission. She denied fever, chest
pain, diaphoresis, hemoptysis and tarry stool passage.
Auscultation revealed a grade II/VI pansystolic murmur
over the left lower sternal border. There was no hepatomegaly or leg edema, but cyanosis with clubbing of
fingers and toes was observed.
Initial evaluation at emergency department revealed
severe hypoxia (the oxygen saturation was 67.9% by ar-
Received: July 23, 2007
Accepted: September 10, 2007
Division of Cardiology, Chang Gung Memorial Hospital-Kaohsiung
Medical Center, Chang Gung University College of Medicine, Taiwan.
Address correspondence and reprint requests to: Dr. Chih-Yuan Fang,
Division of Cardiology, Chang Gung Memorial Hospital-Kaohsiung
Medical Center, 123, Ta Pei Road., Niao Sung Hsiang, Kaohsiung
Hsien 833, Taiwan, R.O.C. Tel: 886-7-731-7123 ext. 2363; Fax:
886-7- 731-7123 ext. 2355; E-mail: [email protected]
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Ming-Chien Wu et al.
rents, percutaneous ASD closure was attempted. Under
general anesthesia and TEE monitoring, a right femoral
venous approach was performed with a 6-French sheath.
A J-curve wire was advanced from the right atrium through the ASD to the left upper pulmonary vein with the
guidance of a 6-French Judkins Right 4 catheter. The venous sheath was replaced with an 8-French Amplatzer
delivery sheath, and the tip of the catheter was advanced
to the left atrium. Under TEE, one secundum-type ASD
measured 11.4 mm ´ 11.5 mm at 42° and 86° of biatrial
view with bidirectional shunt and another tiny ASD
measured 1.82 mm with right-to-left shunt (Figure 3A).
The distance between these two ASDs was 11 mm. A
15-mm Amplatzer septal occluder was well deployed to
the larger ASD under TEE guidance (Figure 3B-C).
There was no residual shunt through the larger ASD.
The tiny ASD shunt remained (Figure 3D). There was
neither damage to the adjacent valve nor obstruction of
the blood flow. The patient tolerated the procedure well
without complication. Her systemic arterial oxygen rose
from 81% to 97% immediately after ASD closure, and
no significant hemodynamic changes occurred during
the procedure. Mean right atrial pressure did not change
obviously (from 6 mmHg to 7 mmHg).
On the next day, follow-up TTE study confirmed the
stable position of the device. The oxygen saturation was
95.9% by arterial blood gas analysis. No significant residual shunt or significant tricuspid regurgitation were
found by TTE one month later. The patient did well with
improved exercise capacity and cyanosis during the subsequent 6 months. No tachyarrhythmia episodes occurred after ablation. However, we will continue to mo-
terial blood gas analysis) and supraventricular tachycardia with hypotension that needed cardioversion. The
electrocardiogram after cardioversion showed ventricular pre-excitation. Transthoracic echocardiography (TTE)
and transesophageal echocardiography (TEE) (Figure 1)
showed downward displacement of the tricuspid valve,
atrialized RV and secundum-type ASD with bi-directional shunt, which was compatible with Ebstein’s anomaly. There was mild tricuspid regurgitation.
Cardiac catheterization of the right-side heart showed left atrium oxygen saturation step-down. The oxygenation series disclosed interatrial communication with
bidirectional shunt (the oxygen saturation was 59% in
the right atrium, 59% in the right ventricle, 59% in the
main pulmonary artery, 97% in the pulmonary vein, 81%
in the left atrium, 84% in the left ventricle, and 81% in
the aorta). The pulmonary artery pressure was 14/7
mmHg, and pulmonary capillary wedge pressure was 10
mmHg. While temporally occluding the ASD using a
sizing balloon, the systemic oxygen saturation increased
from 81% to 98% gradually over 10 minutes. Cyanosis
improved and the patient tolerated ASD closure well. No
deteriorated hemodynamic changes occurred. The blood
pressure was around 102/64 mmHg.
Later, electrophysiologic study was performed for
Wolff-Parkinson-White syndrome, and the patient experienced hypotension when supraventricular tachycardia
was induced. Two accessory (right posterior and right
lateral) pathways were identified and ablated successfully (the electrocardiograms before and after ablation
are shown in Figure 2).
After obtaining consent from the patient and her pa-
Figure 1. (A) Four-chamber view of transthoracic echocardiography revealed apical displacement of the tricuspid valve and atrialized right
ventricle. (B) Left-to-right shunt from the atrial septal defect (ASD) by transesophageal echocardiography. (C) Right-to-left shunt from the ASD.
Acta Cardiol Sin 2008;24:51-5
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Amplatzer ASD Occluder in Ebstein’s Anomaly
Figure 2. The 12-lead surface complete electrocardiograms before (A) and after (B) ablation of accessory pathways.
Figure 3. (A) Another tiny atrial septal defect (ASD) was detected by transesophageal echocardiography (TEE) during the procedure. (B)
Transcatheter closure of ASD using Amplatzer septal occluder (ASO) under TEE monitoring (C) ASO was well deployed with stable position. (D) The
tiny ASD shunt remained after closing the larger ASD but disappeared one month later by transthoracic echocardiography follow-up.
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Ming-Chien Wu et al.
tion immediately, prevent desaturation during exercise,
and improve daily work capacity. 10 Since the transcatheter closure of ASD is feasible by the Amplatzer
septal occluder,12 it provides a non-surgical method to
mitigate the patient’s symptoms. Agnoletti et al 8 reported the successful transcatheter closure of interatrial
shunt in patients with Ebstein’s anomaly. All of these patients had the minor form of Ebstein’s disease, with only
symptoms of desaturation. Only one patient had age similar to our patient’s. None had significant changes of
tricuspid valve regurgitation after ASD closure. The closure of the interatrial communication is not thought to
prevent a possible surgical option in the future. Indeed,
the risk of repeated stroke is considered important.8
In conclusion, patients with Ebstein’s anomaly often
develop systemic desaturation and risk of systemic embolization due to right-to-left shunting. Before deterioration of right-side heart function and tricuspid regurgitation, early intervention to repair the interatrial communication might improve outcomes. In our report, transcatheter Amplatzer septal occluder for Ebstein’s anomaly concomitant ASD is a feasible and safe way. The
patient got significant improvement of cyanosis and exercise intolerance after closure of interatrial shunting.
Although the long-tern outcome may need more time to
observe, the immediate and short-term results are quite
encouraging.
nitor this patient for long-term outcome.
DISCUSSION
Ebstein’s anomaly is an unusual congenital heart
disease with variable clinical presentation. The characteristic anatomic feature is downward displacement and
adherence of dysplastic septal and posterior tricuspid
leaflets into the right ventricle. The right ventricle is divided into a so-called atrialized chamber and a functionally reduced right ventricle. 1-2 Patients with Ebstein’s
anomaly have a high potential for developing arrhythmia. Most of these tachyarrhythmias are based on accessory pathways located along the anomalous atrioventricular valve.6 A single accessory pathway was present
in 52% and multiple accessory pathways in 29% of
cases. Ablation for accessory pathway has initial success
in 79~89% of cases; however, the recurrence rate is
29~32%.9
Our patient had Ebstein’s anomaly without any treatment till adolescence. She had only mild symptoms with
exercise intolerance and cyanosis. So, she didn’t have
heart failure sign in the past. She was admitted because
of newly experienced supraventricular tachycardia. After
electrophysiologic study, catheter ablation was performed for the tachyarrhythmia successfully. The further
therapeutic consideration for this patient is how to improve her life quality.
In fact, the right-to-left shunting in the congenital
heart disease is associated with two major complications: systemic embolization and desaturation. The cerebral events are mostly in the presence of a right-to-left
shunting through the atrial septum. Percutaneous closure
of patent foramen ovale is considered at least as effective as medical treatment in patients with cryptogenic
stroke and recurrent cerebrovascular events.10 Chen et al
reported that earlier intervention in Ebstein’s anomaly
should be considered before the development of significant deterioration in right-side heart function and tricuspid regurgitation. The early results for surgical repair
of Ebstein’s anomaly are quite good.11
The development of symptomatic dyspnea or cyanosis is related to right-to-left shunting or decline in
right ventricular function. Closure of such shunt will be
able to promote the increase of systemic oxygen saturaActa Cardiol Sin 2008;24:51-5
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Amplatzer ASD Occluder in Ebstein’s Anomaly
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