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

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 fo-

ramen 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, arrhyth-

mia, and pulmonary hypertension due to right ventricle

overloads. Closure of interatrial communication might

prevent, improve or resolve these conditions. In the cur-

rent era, transcatheter closure can be performed safely

and successfully.7 Percutaneous closure of Ebstein’s ano-

maly-associated ASD is rarely reported.8 We report our

experience of transcatheter closure of ASD using Am-

platzer septal occluder in a case with Ebstein’s anomaly.

CASE REPORT

A 25-year-old lady had uncertain diagnosed heart

disease since the age of six. She had exercise intoler-

ance, easy fatigue and cyanosis of nail bed since child-

hood. However, she hadn’t experienced orthopnea, par-

oxysmal 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 hepa-

tomegaly 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-

51 Acta Cardiol Sin 2008;24:51�5

Case Reports Acta Cardiol Sin 2008;24:51�5

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: cyfang@seed.net.tw

terial blood gas analysis) and supraventricular tachycar-

dia with hypotension that needed cardioversion. The

electrocardiogram after cardioversion showed ventricu-

lar 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-direc-

tional shunt, which was compatible with Ebstein’s anom-

aly. There was mild tricuspid regurgitation.

Cardiac catheterization of the right-side heart show-

ed left atrium oxygen saturation step-down. The oxygen-

ation 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 expe-

rienced hypotension when supraventricular tachycardia

was induced. Two accessory (right posterior and right

lateral) pathways were identified and ablated success-

fully (the electrocardiograms before and after ablation

are shown in Figure 2).

After obtaining consent from the patient and her pa-

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 th-

rough the ASD to the left upper pulmonary vein with the

guidance of a 6-French Judkins Right 4 catheter. The ve-

nous 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 re-

sidual shunt or significant tricuspid regurgitation were

found by TTE one month later. The patient did well with

improved exercise capacity and cyanosis during the sub-

sequent 6 months. No tachyarrhythmia episodes oc-

curred after ablation. However, we will continue to mo-

Acta Cardiol Sin 2008;24:51�5 52

Ming-Chien Wu et al.

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.

53 Acta Cardiol Sin 2008;24:51�5

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.

nitor this patient for long-term outcome.

DISCUSSION

Ebstein’s anomaly is an unusual congenital heart

disease with variable clinical presentation. The charac-

teristic anatomic feature is downward displacement and

adherence of dysplastic septal and posterior tricuspid

leaflets into the right ventricle. The right ventricle is di-

vided into a so-called atrialized chamber and a function-

ally reduced right ventricle.1-2 Patients with Ebstein’s

anomaly have a high potential for developing arrhyth-

mia. Most of these tachyarrhythmias are based on acces-

sory pathways located along the anomalous atrioven-

tricular 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 treat-

ment 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 per-

formed for the tachyarrhythmia successfully. The further

therapeutic consideration for this patient is how to im-

prove her life quality.

In fact, the right-to-left shunting in the congenital

heart disease is associated with two major complica-

tions: systemic embolization and desaturation. The cere-

bral 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 effec-

tive 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 signifi-

cant deterioration in right-side heart function and tri-

cuspid regurgitation. The early results for surgical repair

of Ebstein’s anomaly are quite good.11

The development of symptomatic dyspnea or cya-

nosis 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 satura-

tion immediately, prevent desaturation during exercise,

and improve daily work capacity.10 Since the trans-

catheter 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 al8 re-

ported the successful transcatheter closure of interatrial

shunt in patients with Ebstein’s anomaly. All of these pa-

tients had the minor form of Ebstein’s disease, with only

symptoms of desaturation. Only one patient had age si-

milar to our patient’s. None had significant changes of

tricuspid valve regurgitation after ASD closure. The clo-

sure 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 em-

bolization due to right-to-left shunting. Before deteriora-

tion of right-side heart function and tricuspid regurgita-

tion, early intervention to repair the interatrial communi-

cation might improve outcomes. In our report, trans-

catheter Amplatzer septal occluder for Ebstein’s ano-

maly concomitant ASD is a feasible and safe way. The

patient got significant improvement of cyanosis and ex-

ercise 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.

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55 Acta Cardiol Sin 2008;24:51�5

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