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Fig. 10 ICE image of
measurement of aortic
and posterior rims of
ASD in the 45°
mid-esophageal view
Fig. 11 ICE image of
measurement of mitral
and atrial rims of ASD
in the 0° mid-esophageal
view
A. Desai et al.
tors for erosion with the Amplatzer device are:
decient rims (particularly inferior), a balloon
sized stop ow diameter 5mm greater than the
static diameter or a low weight to device size
ratio [34]. Given the design of the Gore device,
these rims may not be as necessary for erosion
risk but tissue rim is needed for device stability. A
decient rim from the defect to the AV valves can
lead to AV valve dysfunction. There are many dif-
ferent techniques and tricks described to optimally place these devices in complex anatomy
that are not covered in this chapter given its focus
on imaging. In fact, there have been cases in
which multiple devices can be used to close large
defects (Fig.18) where the rst device is left in
place and a second device from a second access
site is deployed, essentially using the rst as the
“rim” for the second.

Patent Foramen Ovale andAtrial Septal Defect
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Fig. 12 Biplane TEE
image of balloon
crossing interatrial
defect while balloon
sizing is performed
Fig. 13 3D TEE image
of the delivery catheter
advanced into the LA to
ensure the catheter is in
the left upper pulmonary
vein
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Fig. 14 3D TEE image of LA disc deployed and pulled against the septum
A. Desai et al.
Fig. 15 3D TEE image of the RA disc is deployed
Fig. 16 Biplane ICE images with color Doppler are obtained during tug test to ensure no signicant leaks or impinge-
ment on the surrounding structures: SVC and IVC ow, mitral valve function and the aortic root before release

Patent Foramen Ovale andAtrial Septal Defect
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Fig. 17 Biplane ICE images with color Doppler after device is released and further evaluation for leaks is performed
Fig. 18 ICE image of
large ASD with rst
device is left in place
and a second device
from a second access
site is deployed,
essentially using the rst
as the “rim” for the
second
279
Post Procedural Assessment
ASD and PFO: After the procedure, a transthoracic echocardiography is performed to document
the position of the device and a bubble study to
document any residual shunting (Video 14).
Though not in this case, in many cases, a tiny shunt
remains, especially with provocative maneuvers.
This should not by itself be a cause for alarm as
most times, with endothelialization, the follow up
TTE shows no residual shunting. It often takes a
full year to be able to assess the nal degree of
closure because endothelialazation is a slow process. Additionally, the echo should be reviewed for
any sign of pericardial uid as this may be an early
sign of erosion or intra-procedural injury of cardiac chambers. If new pericardial uid is noted, it
may be worth keeping the patient overnight for a
repeat echo in the morning instead of discharging
them after recovery. Post-PFO closure, recommendations are usually for at least 1month of dual
anti-platelet therapy following by indenite low
dose aspirin therapy in patients with prior stroke
[12]. SBE prophylaxis is recommended for a minimum of 6months. In patients closed for non-stroke
indications such as right sided chamber dilation or
platypnea-orthodeoxia, our practice is to stop aspirin after 6months.
Repeat imaging usually consists of another
TTE and bubble study sometime between 3 and

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A. Desai et al.
6 months post procedure. After that, routine
imaging is not recommended unless there are
related complications such as new strokes, infection, or new arrhythmias.
new clinical events that would suggest device
Multimodality imaging comparison
Modality Degree of shunting Anatomic characterization Procedural guidance Post procedure
TTE +++ + ++ ++
TEE +++ +++ +++ +++
ICE +++ +++ +++
MRI ++ (ASD) + (PFO), ++ (ASD)
CT
Angiography + + +
− − (PFO), ++ (ASD) −
Clinical Controversies andPearls
• The presence of anatomic “high risk” features,
such as atrial septal aneurysm and “large”
shunt (dened as greater than 20 bubbles seen
in the left atrium on TEE imaging) increase
the likelihood of recurrent stroke and are
predictive of benet from closure in patients
with PFO and cryptogenic stroke
• Device selection for PFO closure is based on
operator experience and device availability as
randomized head to head data are lacking. The
cardioform device may be preferred in the set-
−
successfully with intra-cardiac echocardiography or TEE
– ASD guidance may be better with TEE
given the higher degree of complexity of
the closure procedure as well as need for
accurate characterization of tissue rims
which may not always be seen with 2D
ICE
– CT and MRI are helpful to evaluate true
atrial septal defects but are limited in the
evaluation of patent foramen ovale due
to the dynamic nature of shunting
−
++
++
−
ting of decient atrial rims due to a perceived
lower risk of device erosion
• For ASDs, cardiac MRI may be considered as
Chapter Review Questions
part of the primary evaluation due to the ability to assess chamber size, pulmonary vein
and great vessel anatomy, as well as cardiac
hemodynamics (Qp/Qs, shunt fraction, cardiac output) ion a single exam, and provide
3D reconstructions for 3D printing or other
applications in a single exam.
1. A 25-year-old woman presents with cryptogenic stroke. Which of the following ndings
is not typical for patent foramen ovale?
A. Positive bubble study at rest that increases
with Valsalva
B. Right ventricular dilation
Key Points
– TTE with bubble study is the rst step in
evaluation of right to left shunting, however, the use of lower extremity injection
and Valsalva may be needed to increase
the sensitivity of the bubble study
– TEE is the preferred modality for the ana-
tomic characterization of inter-atrial
shunts
– Procedural guidance can be achieved
C. Bubble study only positive with Valsalva
D. Inter-atrial septal aneurysm
Answer: B
Explanation: As patent foramen ovales are
ap-like, they typically are only associated
with right to left shunting though a small
amount of bidirectional shunting can be seen
when “stretched” open. If right ventricular
dilation, pulmonary hypertension, or right
atrial dilation are seen, suspicion for an atrial
septal defect or congenital abnormalities

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281
should increase. Of note, inter-atrial septal
aneurysms are commonly seen in PFOs and
are an imaging marker of high risk of stroke
recurrence. Bubble studies may be positive
either at rest, or with Valsalva, or both, depending on the relative RA and LA pressures.
2. A 40-year-old woman presents with symptoms of dyspnea without hypoxemia and right
ventricular enlargement is seen on TTE as
well as color ow Doppler of the inter-atrial
septum showing left to right shunting. Which
defect is statistically most likely?
A. Patent foramen ovale
B. Septum primum
C. Septum secundum
D. Sinus venosus
E. Unroofed coronary sinus
Answer: C
Explanation: Septum secundum defects
are the most common type of atrial septal
defect. Patent foramen ovale would not typically be associated with dyspnea unless
hypoxemia is seen.
3. A 78-year-old man presents with severe
hypoxemia upon standing and with exertion.
He has no history of lung disease and PFTs
are normal. A TTE performed with agitated
saline in an antecubital vein shows complete
white out of the left sided chambers within
two heart beats of the bubbles arriving in the
right atrium. Which concomitant abnormality
should be ruled out?
A. Aortic aneurysm
B. Cirrhosis
C. Arterio-venous stula
D. Lung cancer
Answer: A
Explanation: Aortic aneurysms can cause
shifting of the inter-atrial septum leading to
opening of a previously closed patent foramen
ovale. B and C would be suggested by the late
arrival of bubbles in the left atrium.
4. A 25-year-old man presents with cryptogenic
stroke. TTE bubble study is negative at rest.
Due to a high index of suspicion, a TEE is
performed and a PFO with inter-atrial septal
aneurysm is found. Additionally, a prominent
eustachian valve is noted. Which maneuvers
could have discovered the PFO on the initial
TTE?
A. Injection of a higher volume of agitated
saline
B. Lower extremity injection
C. Valsalva
D. Use of echo microbubble contrast
Answer: B and C
Explanation: Both Valsalva maneuvers
and lower extremity injection can increase the
sensitivity of agitated saline microbubble
studies. Generally, the initial TTE should
always include provocative maneuvers. Lower
extremity injections are rarely needed.
5. A 38-year-old woman underwent closure of
an inter-atrial shunt of unknown type 2years
prior with an unknown device in the setting of
an embolic stroke. She presents with cardiac
tamponade and bloody uid is removed. A
TEE reveals hematoma surrounding the aortic
root. A device is seen but poorly visualized.
Which of the following factors is least likely
to be involved?
A. Decient aortic rim
B. Gore Cardioform PFO Occluder 30mm
C. Amplatzer PFO Occluder 35mm
D. Amplatzer ASO Occluder
Answer: B
Explanation: Decient aortic rims are
associated with increased risk of erosion with
Amplatzer type devices. The frequency of
erosion is one case per several thousand
implants of the Amplatzer ASO device and is
even more rare with larger Amplatzer PFO
occluders.
References
1. Gill EA.Denitions and pathophysiology of the patent foramen ovale: broad overview. Cardiol Clin.
2005;23(1):1–6.
2. Bannan A, et al. Characteristics of adult patients
with atrial septal defects presenting with paradoxical embolism. Catheter Cardiovasc Interv.
2009;74(7):1066–9.
3. Hagen PT, Scholz DG, Edwards WD. Incidence
and size of patent foramen ovale during the rst 10
decades of life: an autopsy study of 965 Normal
hearts. Mayo Clin Proc. 1984;59(1):17–20.

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4. Surkova E, etal. International journal of cardiology
congenital heart disease the ACHD multi-modality
imaging series: imaging of atrial septal defects in
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5. Gill EA, Quaife RA. The echocardiographer and
the diagnosis of patent foramen ovale. Cardiol Clin.
2005;23(1):47–52.
6. Radico F, et al. The ‘dreaded PFO’: anatomical and
functional features of high risk for stroke. Eur Heart J
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7. Nakayama R, et al. Identication of high-risk patent foramen ovale associated with cryptogenic
stroke: development of a scoring system. J Am Soc
Echocardiogr. 2019;32(7):811–6.
8. Kumar P, Rusheen J, Tobis JM. A comparison of
methods to determine patent foramen ovale size.
Catheter Cardiovasc Interv. 2020;96(6):E621–9.
9. Kent DM, etal. An index to identify stroke-related vs
incidental patent foramen ovale in cryptogenic stroke.
Neurology. 2013;81(7):619–25.
10. Kent DM, et al. Risk of paradoxical embolism
(RoPE)-estimated attributable fraction correlates with
the benet of patent foramen ovale closure: an analysis of 3 trials. Stroke. 2020;51(10):3119–23.
11. Kent DM, et al. Heterogeneity of treatment effects
in an analysis of pooled individual patient data from
randomized trials of device closure of patent foramen
ovale after stroke. JAMA. 2021;326(22):2277–86.
12. Kavinsky CJ, etal. SCAI guidelines for the management of patent foramen ovale. J Soc Cardiov Angiogr
Interv. 2022;1:100039.
13. Saver JL, et al. Long-term outcomes of patent foramen ovale closure or medical therapy after stroke. N
Engl J Med. 2017;377(11):1022–32.
14. Kasner SE, etal. Patent foramen ovale closure with
GORE HELEX or CARDIOFORM septal Occluder
vs. antiplatelet therapy for reduction of recurrent stroke
or new brain infarct in patients with prior cryptogenic
stroke: design of the randomized Gore REDUCE clinical study. Int J Stroke. 2017;12(9):998–1004.
15. Søndergaard L, etal. Patent foramen ovale closure or
antiplatelet therapy for cryptogenic stroke. N Engl J
Med. 2017;377(11):1033–42.
16. Mojadidi MK, et al. Transcatheter patent foramen
ovale closure after cryptogenic stroke. JACC Cardiov
Interv. 2017;10(21):2228–30.
17. Chen GP-W, Goldberg SL, Gill EA Jr. Patent foramen ovale and the platypnea-orthodeoxia syndrome.
Cardiol Clin. 2005;23(1):85–9.
18. Mojadidi MK, etal. The effect of patent foramen ovale
closure in patients with platypnea-orthodeoxia syndrome. Catheter Cardiovasc Interv. 2015;86(4):701–7.
19. Dowson A, et al. Migraine intervention with
STARFlex technology (MIST) trial. Circulation.
2008;117(11):1397–404.
20. Mojadidi MK, etal. Pooled analysis of PFO Occluder
device trials in patients with PFO and migraine. J Am
Coll Cardiol. 2021;77(6):667–76.
21. Mattle HP, etal. Percutaneous closure of patent foramen ovale in migraine with aura, a randomized controlled trial. Eur Heart J. 2016;37(26):2029–36.
22. Ahmed Z, Sommer RJ.Reassessing the PFO-migraine
trials: are we closer to closure? J Am Coll Cardiol.
2021;77(6):677–9.
23. Faccini A, Butera G. Atrial septal defect (ASD)
device trans-catheter closure: limitations. J Thorac
Dis. 2018;10(S24):S2923–30.
24. Poommipanit P, Amin Z.Considerations for ASD closure. Understanding the devices and proper anatomic
evaluation to prevent and manage possible complications. Cardiac Interv Today. 2014:30–9.
25. Wiktor DM, Carroll JD. ASD closure in structural
heart disease. Curr Cardiol Rep. 2018;20(6):37.
26. Thomson JDR, Qureshi SA.Device closure of secundum atrial septal defect’s and the risk of cardiac erosion. Echo Res Pract. 2015;2(4):R73–8.
27. Spina R, etal. Nickel hypersensitivity reaction following Amplatzer atrial septal defect occluder device
deployment successfully treated by explantation of
the device. Int J Cardiol. 2016;223:242–3.
28. Wahl A, et al. Safety and feasibility of percutaneous closure of patent foramen ovale without intraprocedural echocardiography in 825 patients. Swiss
Med Wkly. 2008;138(39):567.
29. Siddiqui IF, Michaels AD.Percutaneous patent foramen ovale closure using Helex and Amplatzer devices
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J Interv Cardiol. 2011;24(3):271–7.
30. Alqahtani F, et al. Intracardiac versus transesophageal echocardiography to guide transcatheter closure of interatrial communications: nationwide
trend and comparative analysis. J Interv Cardiol.
2017;30(3):234–41.
31. Vigna C, etal. Echocardiographic guidance of percutaneous patent foramen ovale closure: head-to-head
comparison of transesophageal versus rotational
intracardiac echocardiography. Echocardiography.
2012;29(9):1103–10.
32. Moon J, etal. Comparison of intracardiac echocardiography and transesophageal echocardiography for
image guidance in percutaneous patent foramen ovale
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33. Kim SS, etal. The use of intracardiac echocardiography and other intracardiac imaging tools to guide noncoronary cardiac interventions. J Am Coll Cardiol.
2009;53(23):2117–28.
34. Mcelhinney DB, etal. Relative risk factors for cardiac
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defects. Circulation. 2016;133(18):1738–46.

Percutaneous Ventricular Septal
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Defect Closure
KamelShibbani, KarimA.Diab, DamienKenny,
andZiyadM.Hijazi
Abstract
Percutaneous Ventricular Septal Defect (VSD)
closure has become a viable alternative to surgery for certain perimembranous and most
muscular ventricular septal defects. Various
devices exist that cater to the unique anatomical variations in each patient. Given the variability in size and location of such defects, a
detailed anatomical assessment is vital to
allow the care team to make the most appropriate decision regarding surgical vs percutaneous VSD closure. Transthoracic
echocardiography is essential in preprocedural
planning, with transesophageal echocardiog-
Supplementary Information The online version contains supplementary material available at https://doi.
org/10.1007/978- 3- 031- 50740- 3_11.
raphy playing an equally important intraprocedural role. Rarely, advanced cross-sectional
imaging with a CT/MRI might be helpful in
complex VSDs. In this chapter, we look at the
standard transthoracic and transesophageal
imaging assessment for percutaneous closure
of perimembranous and muscular VSDs using
a case-based illustrated approach.
Keywords
Percutaneous · Ventricular septal defect ·
Perimembranous · Muscular ·
Transesophageal · Transthoracic ·
Echocardiography
K. Shibbani
Division of Cardiology, Department of Pediatrics,
Rady Children’s Hospital, San Diego, CA, USA
K. A. Diab (*)
Division of Cardiology, Department of Pediatrics,
Lurie Children’s Hospital, Northwestern Feinberg
School of Medicine, Chicago, IL, USA
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024
A. M. Kelsey et al. (eds.), Cardiac Imaging in Structural Heart Disease Interventions,
https://doi.org/10.1007/978-3-031-50740-3_11
Division of Cardiology, Department of Pediatrics,
Inova Children’s Hospital, Fairfax, VA, USA
D. Kenny
Department of Pediatric and Congenital Cardiology,
Children’s Health Ireland at Crumlin, Dublin, Ireland
Z. M. Hijazi
Sidra Heart Center, Sidra Medicine, Weill Cornell
Medicine, Doha, Qatar
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Abbreviations
CT Computed tomography
ECG Electrocardiography
LAX Long axis
MPA Main pulmonary artery
MRI Magnetic resonance imaging
mVSD Muscular ventricular septal defect
pmVSD Perimembrenous ventricular septal
defect
RA Right atrium
RV Right ventricle
RVOT Right ventricular outow tract
SAX Short axis
TEE Transesophageal echocardiography
TTE Transthoracic echocardiography
VSD Ventricular septal defect
Test your learning and check your under-
standing of this book’s contents: use the
“Springer Nature Flashcards” app to
access questions using ▶ https://sn.pub/
ambACS.
To use the app, please follow the instruc-
tions in the chapter “Transcatheter
Aortic Valve Replacement.”
Learning Objectives
1. Identify the best transthoracic echocardio-
graphic imaging planes to assess perimembranous and muscular ventricular septal
defects
2. Identify the best transesophageal echocardio-
graphic imaging planes to assess perimembranous and muscular ventricular septal
defects
3. Identify essential intra-operative and post-
procedural echocardiographic checklists
4. Understand the inclusion and exclusion crite-
ria for percutaneous VSD device closure, especially those identied by echocardiography
Muscular VSD
Case Study
A 31-year-old male involved in a motorcycle accident was admitted in severe hypovolemic shock. Workup included an ECG
that revealed a right bundle branch block
and a transthoracic echo that revealed a
large apical muscular VSD that measured
about 18mm in diameter. Echocardiography
also revealed an avulsed tricuspid valve
with severe regurgitation. The defect had a
gradient of 55 mmHg with left to right
shunting (Qp:Qs was 2:1 on hemodynamic
assessment).
Background andDenitions
A muscular VSD is a defect in the interventricular septum that has exclusive muscular borders.
Muscular VSDs represent the second most common type of VSDs in children, accounting for
approximately 10–15% of such defects [1]. They
are less common in adults but can be seen after
blunt chest trauma [2], as described herein. These
defects are categorized according to their location as being mid muscular vs apical (in relation
to the moderator band), and anterior vs posterior.
They can exist as a single defect, or as multiple
simultaneous defects (Swiss-cheese type of VSD,
when consisting of 4 or more defects) [3].
Diagnosis andPre-procedural
Assessment
Preprocedural evaluation is of paramount importance to determine not just eligibility for percutaneous closure of a mVSD, but also to dene the
characteristics of the VSD and to plan for the best
percutaneous approach, as well as to anticipate
any potential post-procedural complications.
Evaluation begins with a transthoracic echocardiogram (TTE) to identify the location, number,
and size of mVSDs, and to determine the pres-

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ence or absence of any associated cardiac defects.
Pre-procedural TTE also plays an important role
in evaluating the hemodynamic signicance of
mVSD through assessing the size of the left heart
chambers and helps in estimating the amount of
shunt (Qp:Qs). It is worth noting that most small
mVSDs are often not hemodynamically signicant. These small mVSDs are sometimes difcult
to visualize on TTE by 2D and may necessitate
color Doppler evaluation. Important views to
obtain by TTE for the assessment of mVSD
include the parasternal short and long axis views
and the 4-chamber view. The parasternal short
axis view with a sweep beginning at the base of
the heart and progressing to the apex is of particular importance in evaluating mVSDs. In this
view, posterior mVSDs will appear between 7
and 10 o’clock, mid-muscular VSDs will appear
between 10 and 12 o’clock, and anterior mVSDs
will appear between 12 and 2 o’clock [4]. The
parasternal long axis view is also helpful to assess
the location and size of mVSD with particular
sweeps across the septum using color Doppler.
Additional views to assess mVSD location and
number include the apical 4-chamber sweep. In
the apical view, visualization of the defect at the
level of the atrioventricular valves indicates a
posterior mVSD, whereas visualization at the
level of the outow tracts indicates an anterior
mVSD.In addition, the location along the long
axis plane (apical vs mid vs basal) can be interrogated in this view. These various views are also
essential to assess for left sided chamber dilatation and estimating the gradient across the VSD
shunt. In younger patients and children, the subcostal sagittal view is also helpful to assess the
mVSD shunt and location.
Transesophageal echocardiography (TEE)
plays an important intraprocedural role during
mVSD closure. TEE can help with accurate sizing, localization of the defect (s), and identication of total number of defects prior to closure. It
can also be used to monitor closure through
assessing the stability of the device, impingement
on surrounding structures, and residual shunts
post device deployment. TEE views during percutaneous VSD closure include a trans-gastric
short axis view of the left ventricle, mid-
esophageal four chamber view, and the transgastric basal short axis view [5, 6]. These views
are essential to evaluate the shunt location and
size during the intra-operative procedure and for
selecting which VSD to approach rst especially
in the setting of multiple or Swiss Cheese
mVSDs. TEE also provides accurate measurement of the size of the mVSD which is usually
done by 2D and color in order to decide on the
size of the device needed. After other associated
abnormalities are studied and after chamber sizes
and function are assessed, more imaging is performed concentrating on the VSD and nearby
structures, namely, the papillary muscles, moderator band, and the chordae tendinae. The atrioventricular valves are interrogated at baseline for
any regurgitation.
The VSD is measured in multiple views
including the frontal 4-chamber and basal shortaxis views. Tissue rims and distances from aortic and tricuspid valves are also measured in the
above views to determine adequacy for device
closure. The appropriate device size is usually
chosen to be 1–2mm larger than the VSD size
as assessed by TEE with color Doppler and
angiographic evaluation (maximal size at enddiastole). During the closure procedure, the
TEE mid- esophageal 4-chamber view is a helpful home view to help guide passage of the
guidewire and the delivery system across the
defect into the LV cavity and to monitor the subsequent deployment of the LV disk followed by
aligning the device in the appropriate position
against the ventricular septum. This real-time
monitoring of the device by TEE is essential to
help the operator maneuver or reposition the
device when needed and for avoiding any damage to close-by structures such as the mitral, tricuspid and aortic valves. It is also essential to
check for any residual shunting and if signicant then to allow the operator to redeploy a
larger device if needed.
Heart Team Approach andDiscussion
Multiple surgical approaches for mVSD closure have been reported including staged repair
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