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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: decient rims (particularly inferior), a balloon sized stop ow diameter 5mm 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 decient 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 andAtrial 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
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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 signicant leaks or impinge-
ment on the surrounding structures: SVC and IVC ow, mitral valve function and the aortic root before release
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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
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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 1month of dual anti-platelet therapy following by indenite low dose aspirin therapy in patients with prior stroke [12]. SBE prophylaxis is recommended for a mini­mum of 6months. In patients closed for non-stroke indications such as right sided chamber dilation or platypnea-orthodeoxia, our practice is to stop aspi­rin after 6months.
Repeat imaging usually consists of another TTE and bubble study sometime between 3 and
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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 andPearls
• The presence of anatomic “high risk” features, such as atrial septal aneurysm and “large” shunt (dened as greater than 20 bubbles seen in the left atrium on TEE imaging) increase the likelihood of recurrent stroke and are predictive of benet 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 decient 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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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 2years 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. Decient aortic rim B. Gore Cardioform PFO Occluder 30mm C. Amplatzer PFO Occluder 35mm D. Amplatzer ASO Occluder
Answer: B Explanation: Decient 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.Denitions 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, etal. International journal of cardiology congenital heart disease the ACHD multi-modality imaging series: imaging of atrial septal defects in adulthood. Int J Cardiol Congenital Heart Disease. 2021;4:100188.
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 Suppl. 2021;23(Supplement_E):E189–93.
7. Nakayama R, et al. Identication 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, etal. 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 benet 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, etal. 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, etal. 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, etal. 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, etal. 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, etal. 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, etal. 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, etal. 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 without Intraprocedural echocardiographic guidance. 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, etal. 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, etal. Comparison of intracardiac echocar­diography and transesophageal echocardiography for image guidance in percutaneous patent foramen ovale closure. Medicina. 2020;56(8):401.
33. Kim SS, etal. 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, etal. Relative risk factors for cardiac erosion following transcatheter closure of atrial septal defects. Circulation. 2016;133(18):1738–46.
Percutaneous Ventricular Septal
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Defect Closure
KamelShibbani, KarimA.Diab, DamienKenny, andZiyadM.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 outow 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 identied 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 18mm 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 andDenitions
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 andPre-procedural Assessment
Preprocedural evaluation is of paramount impor­tance to determine not just eligibility for percuta­neous closure of a mVSD, but also to dene 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 signicance 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 signi­cant. These small mVSDs are sometimes difcult 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 outow 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 identica­tion 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 1–2mm 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 signi­cant then to allow the operator to redeploy a larger device if needed.
Heart Team Approach andDiscussion
Multiple surgical approaches for mVSD clo­sure have been reported including staged repair