Case Study
Closure of a multi-fenestrated atrial
septal defect with a deficient retroaortic
rim using a GORE® Septal Occluder
C A S E
S T U D Y
Jochen Grohmann, MD
Department of Congenital Heart Disease,
Heart Center Freiburg University, Germany
Background
A six-year-old girl was referred to our heart center
for interventional closure of a large secundum atrial
septal defect (ASD). Besides this, she was known
to be healthy. She had normal exercise tolerance
without any signs of cardiac failure.
Body weight was 20 kg, body length 118 cm; blood
pressure documented 100 / 60 mmHg, normal
oxygen saturation with 99%, on auscultation a
2 / 6 systolic murmur above the pulmonary ostium,
second heart sound splitted; no hepatomegalia.
On ECG incomplete right bundle branch block was
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observed as a typical finding in significant ASD.
On chest x-ray some degree of cardiomegalia with
prominent hili was described.
The large ASD II with left-to-right shunting, right
atrial and right ventricular enlargement as well
as some degree of pulmonary valve stenosis was
demonstrated by transthoracic echocardiography.
There was the impression that there were two
defects: the larger, more superior defect measured
about 12 mm in diameter, the aortic rim was
deficient, and the total septal length was around
29 mm.
Figure 1. TEE of the
anterosuperior defect.
Diameter 13.5–14 mm
in both planes
(42º [A] vs. 118º [B] on TEE).
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SVC
Figure 2. Balloon sizing
of inferior defect with
a 25 mm NuMED Balloon
in AP view (A) and lateral view
(B) shows a defect measuring
10.3 x 15.3 mm.
Informed consent was obtained from
the parents.
Procedure
The intervention was performed
under general anesthesia and
transesophageal echo-guidance
(TEE). The vein in the right groin was
punctured in a Seldinger technique
introducing a 6 Fr short TERUMO®
Sheath. A 4 Fr MP-catheter with
standard 0.035" wire was used
to cross the atrial septum and to
enter a left pulmonary vein. For
balloon sizing the standard wire
was replaced by a 0.035" AMPLATZ
SUPER STIFF Guidewire.
TEE confirmed a multi-perforated
large ASD with both an inferior
and anterosuperior defect. Native
diameters measured 13.5 x 14 mm
for the anterosuperior defect with
no retroaortic rim (Figure 1), but a
sufficient rim to the SVC. The native
diameter for the inferior defect was
10 x 14 mm and was located at the
lower margin with insufficient rim at
the level of the AV valves.
First the inferior part of the defect
was crossed and balloon sized in
stop-flow technique. The defect
appeared to be oval with 10 x
15,mm in diameter on fluoroscopy
(Figure 2) — and up to 16.7,mm on TEE
(Figure 3). While inflating the balloon
in the inferior defect, the shunt via the
anterosuperior defect was not affected
or reduced (Figure 4). The sizing
balloon was taken out, but a coronary
guidewire was left in place as a marker
for the inferior defect.
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LV
Figure 3. TEE with the wire (orange arrow) passing the inferior
defect (top) with a borderline rim. Balloon sizing (bottom)
shows a defect diameter of 15–17 mm.
Figure 4. Anteriosuperior defect is unaffected by balloon
sizing of the inferior defect.
Then the anterosuperior defect was
crossed coming from the SVC under
TEE guidance. Balloon sizing of the
anterosuperior defect demonstrated a
diameter of 12 x 15,mm (Figure 5).
Total septal length was measured at
31–33,mm.
Due to the insufficient retroaortic and
the shortness of the inferior rim as well
as the desire to cover both defects
with only one occluder, a GORE® Septal
Occluder as a non-self-centering
device was our first choice. Given the
septal length of 30–33,mm, we chose
a 30,mm GORE® Septal Occluder. Our
intention was to implant the device in
the anterosuperior defect, and not into
the inferior, to prevent involving the AV
valves.
The GORE® Septal Occluder was
prepared and introduced over the
wire across the anterosuperior defect.
Due to the multipurpose shape of the
delivery system, deployment of both
GORE® Septal Occluder discs was
challenging, especially on the left side
(Figure 6). As long as the GORE® Septal
Occluder was attached to the system,
significant residual shunting was of concern behind
the aorta, at the level of the SVC and AV valves.
This was speculated to be due to tension from the
delivery catheter, so we progressed towards locking
the device to obtain a tension free assessment.
Stability testing (wiggle) confirmed that the two
discs were separated from each other by the
atrial septum, so we attempted to lock the device.
There was initially a delay in the lock release. To
address this, the delivery system components
were repositioned such that the lock loop was able
to come free of the control catheter resulting in
an excellent position (Figure 7). With the tension
released, the discs configured well to cover both
the anterosuperior and inferior defects with perfect
septal alignment. At the end of the procedure only
a trivial residual anterosuperior shunt remained —
without any hemodynamic relevance (Figure 8). The
GORE® Septal Occluder covered the entire septum
without any interaction with the AV valves or any other
intracardiac structures.
After an uneventful post-interventional observation
period, the patient was discharged with the advice to
take prophylactic aspirin therapy as well as to consider
the recommendations for prevention of endocarditis
for six months.
Follow-up after four weeks, three months and six
months confirmed a stable device position with a
perfect result without any residual shunting. There was
complete closure, including at the level of the SVC.
Figure 5. Balloon sizing of the
anterosuperior defect with a
25 mm NuMED Balloon — 12
x 15 mm in AP view (A) and
lateral view (B).
Figure 6. Residual shunting at
the aorta during deployment.
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Discussion and Conclusion
The GORE® Septal Occluder has a five-wire frame that
supports an ePTFE occlusion membrane uniquely
suitable for device-closure of ASDs. Implantation
was successful in this case, even with an insufficient
retroaortic rim. In multi-fenestrated ASDs, the
GORE® Septal Occluder is ideal for covering multiple
defects and most of the septum with one device. In
conclusion, the GORE® Septal Occluder is easy to
use and was successful in this pediatric patient.
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Figure 7. Final configuration under fluoroscopy.
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Figure 8. TEE after final release demonstrating complete
closure in the region of the deficient anterosuperior rim (top)
and a trivial shunt at the level of the SVC (bottom).
W. L. Gore & Associates, Inc.
Flagstaff, AZ 86004
+65.67332882 (Asia Pacific)
00800.6334.4673 (Europe)
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© 2014 W. L. Gore & Associates, Inc. AT0962-EU2 MARCH 2014