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Fig. 29.14 Leaving the wire in place, the occluder device is
deployed. Next, fluoroscopic and echocardiographic studies
are performed to assess for any possible mechanical effects
on adjacent valvular or other structures. If no such impingement exists, the device can be deployed with its narrowest
diameter across the neck of the pseudoaneurysm.
Fig. 29.15 An angiogram is performed to confirm there is
no leak around the device. Further confirmation is obtained
using TTE, TEE, or ICE. The wire is removed and the device is
released. A repeat biplane angiogram is performed to confirm
complete closure.
Fig. 29.16 Antegrade (transseptal) access may be required in left
atrial or mitral valve pseudoaneurysms. In those cases, we use an
8.5F Agilis sheath along with the telescoping catheters to engage the
pseudoaneurysm. The steps describe earlier are then followed.
29—PSEUDOANEURYSM DIAGNOSIS AND MANAGEMENT 357
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Fig. 29.17 For smaller pseudoaneurysms with a very narrow
neck, endovascular coils can be used to plug the pseudoaneurysm. Care must be exercised to avoid any extension of the
coils into the left ventricle (LV) or aorta. For this approach, initial
steps include femoral artery access, biplane angiogram, selective engagement of the pseudoaneurysm, and placement of the
guiding catheter.
Fig. 29.18 Commercially available endovascular coils can
be placed within the sac to completely obliterate the cavity.
Repeat angiogram is performed to confirm complete closure.
Fig. 29.19 Combined occluder device and coil embolization.
In very large pseudoaneurysms or those with multiple lobes or
incomplete closure with the occluder device alone, a combined
approach can be used. Similar steps are used to engage the
pseudoaneurysm, followed by deployment of the occluder device. A 4F multipurpose catheter is then advanced over the wire
into the pseudoaneurysm and the wire is removed. Endovascular coils are then introduced with complete packing of the sac.
The occluder device is secured in position to make sure there is
no extension or embolization of coil outside the pseudoaneurysm sac.
358 8—ACHD INTERVENTIONS
AB
CD
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Fig. 29.20 Stent-assisted coiling of a coronary aneurysm or pseudoaneurysm. (A) A microcatheter is ad-
vanced into the aneurysm, jailed by a noncovered stent, and followed by coil delivery via the microcatheter. A
final postdilation of the stent is often performed. (B) Coils are delivered through the stent struts after initial stent
deployment. (C) A microcatheter is advanced into the aneurysm, jailed by a coronary balloon, followed by coil
delivery via the microcatheter. A noncovered stent deployment usually follows. (D) Final result of stent-assisted
coil embolization. (Reproduced with permission from Kawsara A, Nunez Gil IJ, Alqahtani F, Moreland J, Rihal
CS, Alkhouli M. Management of coronary artery aneurysms. JACC Cardiovasc Interv. 2018;11(13):1211-1223.)
Surgical Management
Surgical management was considered the gold standard in most centers; however, it was associated
with very high mortality risk (≈ 10% immediately postoperatively, ≈ 20% within 1 week).
death included heart failure, acute MI, and rhythm disturbances. Most of these studies included patients before the development of percutaneous techniques for the management of pseudoaneurysms.
With the development of advanced percutaneous techniques, indications for surgical management
include:
1. Very large pseudoaneurysms with no neck (Fig. 29.22)
2. Pseudoaneurysms unapproachable by current occluder devices because of the risk of
impingement on surrounding structures
3. Failure of percutaneous approach
4. Patient due to undergo another cardiac surgery
1,6
Cause of
Follow-Up
All patients should be followed with repeat imaging within 1 month with CT scan or MRI to
assess the size of the pseudoaneurysm and stability of the implanted devices. If there is enlargement, it may be a sign of incomplete closure, and the patient may need repeat percutaneous
closure of residual neck or cardiac repair.
16
Conclusions
n
Pseudoaneurysms are a contained rupture of the arterial or myocardial wall and an infrequent
complication of acute myocardial complication or a cardiac procedure.
n
Diagnosis may be incidental, or the patients may present with symptoms.
29—PSEUDOANEURYSM DIAGNOSIS AND MANAGEMENT 359
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Fig. 29.21 Covered stent to treat vein graft pseudoaneurysm. (A) Angiogram of saphenous vein
graft to distal right coronary artery (RCA) demonstrated a large pseudoaneurysm measuring 10 cm
3 6 cm. (B) A 7 3 10 cm Viabahn peripheral stent was placed successfully excluding the pseudoanuerysm. (Reproduced with permission from Rahim SA, Pitta SR, Rihal CS. Saphenous vein graft
pseudoaneurysm. J Am Coll Cardiol. 2009;53(20):1918.)
Fig. 29.22 Large calcified pseudoaneurysm not amenable for percutaneous closure. Large calcified
posterobasal ventricular pseudoaneurysm (yellow arrow) disrupting the continuity between the aortic and
mitral annulus. The pseudoaneurysm has a wide neck. The patient was treated successfully with surgical
repair. AV, Mechanical aortic valve; LV , left ventricle; MV, mechanical mitral valve.
n
Pseudoaneurysms of the LV are at a high risk of rupture, so treatment is recommended in
most patients, if feasible. Surgical treatment was the gold standard until recently; however,
it is associated with high in-hospital mortality and morbidity.
n
Percutaneous approaches using occluder devices, coil embolization, covered stents, or
combined approaches should be the standard of care, with a few exceptions as outlined
earlier.
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References
1. Frances C, Romero A, Grady D. Left ventricular pseudoaneurysm. J Am Coll Cardiol. 1998;32(3):557-561.
2. Hulten EA, Blankstein R. Pseudoaneurysms of the heart. Circulation. 2012;125(15):1920-1925.
3. Vlodaver Z, Coe JI, Edwards JE. True and false left ventricular aneurysms. Propensity for the latter to
rupture. Circulation. 1975;51(3):567-572.
4. Brown SL, Gropler RJ, Harris KM. Distinguishing left ventricular aneurysm from pseudoaneurysm.
A review of the literature. Chest. 1997;111(5):1403-1409.
5. Atik FA, Navia JL, Vega PR, et al. Surgical treatment of postinfarction left ventricular pseudoaneurysm.
Ann Thorac Surg. 2007;83(2):526-531.
6. Yeo TC, Malouf JF, Oh JK, Seward JB. Clinical profile and outcome in 52 patients with cardiac
pseudoaneurysm. Ann Intern Med. 1998;128(4):299-305.
7. Langer NB, Hamid NB, Nazif TM, et al. Injuries to the aorta, aortic annulus, and left ventricle during
transcatheter aortic valve replacement: management and outcomes. Circ Cardiovasc Interv. 2017;10(1)
e004735.
8. Sudhakar S, Sewani A, Agrawal M, Uretsky BF. Pseudoaneurysm of the mitral-aortic intervalvular
fibrosa (MAIVF): a comprehensive review. J Am Soc Echocardiogr. 2010;23(10):1009-1018; quiz 1112.
9. Tuan J, Kaivani F, Fewins H. Left ventricular pseudoaneurysm. Eur J Echocardiogr. 2008;9(1):107-109.
10. Al-Hijji MA, Guerrero M, Rihal CS, Eleid MF. Transapical percutaneous closure of rapidly expanding
post-surgical left ventricular outflow tract pseudoaneurysm. Catheter Cardiovasc Interv. 2019;94(6):859-862.
11. Goel K, Foley TA, Warnes CA, McLeod CJ. Pseudoaneurysm development after ablation for atrial
tachycardia in a patient with single-ventricle anatomy. JACC Clin Electrophysiol. 2016;2(4):524-525.
12. Kumar PV, Alli O, Bjarnason H, Hagler DJ, Sundt TM, Rihal CS. Percutaneous therapeutic approaches
to closure of cardiac pseudoaneurysms. Catheter Cardiovasc Interv. 2012;80(4):687-699.
13. Rahim SA, Greason KL, Bjarnason H, Rihal CS. Left ventricular pseudoaneurysm. J Am Coll Cardiol.
2009;54(8):740.
14. Kawsara A, Nunez Gil IJ, Alqahtani F, Moreland J, Rihal CS, Alkhouli M. Management of coronary
artery aneurysms. JACC Cardiovasc Interv. 2018;11(13):1211-1223.
15. Rahim SA, Pitta SR, Rihal CS. S aphenous vein graft pseudoaneurysm. J Am Coll Cardiol. 2009;53(20):1918.
16. Shah S, Maor E, Joyce DL, Rihal CS. Percutaneous closure of subannular rupture following transcatheter
aortic valve implantation. EuroIntervention. 2018;13(13):1536-1537.
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Abstract: Pseudoaneurysms are an uncommon complication of surgical procedures and are
associated with a high risk of complications. The diagnosis is most often made incidentally on
imaging. Multimodality imaging including CT scan, MRI, TEE, and 3D printing is key for
diagnosis and management. Percutaneous closure of pseudoaneurysms can be performed safely
with proper planning and execution. This book chapter describes various percutaneous techniques
for pseudoaneurysm closure.
Keywords: Pseudoaneurysm, percutaneous closure, occluder device, covered stent
CHAPTER 30
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Pulmonary Balloon Angioplasty
for Chronic Thromboembolic
Pulmonary Hypertension
Abdallah El Sabbagh Gurpreet S. Sandhu
Chronic thromboembolic pulmonary hypertension (CTEPH) is a pulmonary vascular disease
that results from fibrotic transformation of thromboemboli causing obstruction in the pulmonary
vasculature. If left untreated, pulmonary artery pressure worsens, affecting right ventricular function and causing significant morbidity and mortality, with up to 68% 5-year survival.
registry data suggest that the annual incidence of CTEPH is about 5 per million adults per year,
which is likely an underestimate due to underrecognition of the disease. This makes it one of the
most common causes of pulmonary hypertension.
Up to 3% of patients with acute pulmonary embolism progress to develop CTEPH.
CTEPH, the pulmonary thromboemboli remain attached to the wall of the pulmonary vasculature, which can be distributed in the main, lobar, segmental, and/or subsegmental branches of the
pulmonary vasculature. This then triggers an inflammatory process leading to organization and
fibrosis of this thrombus into webs, luminal narrowing, and sometimes complete obstruction of
the pulmonary vessels.
4
1
European
3
In
2
Clinical Presentation
Patients with CTEPH present with dyspnea and/or signs of right-sided heart failure. CTEPH
may be suspected when there are persistent symptoms after 3 months of anticoagulation after an
acute pulmonary embolus. This disease can be suspected from the history, including risk factors
for hypercoagulable states, signs, and symptoms, along with ancillary testing, which includes
electrocardiography (showing right ventricular strain pattern), chest radiography, pulmonary
function testing, and echocardiography. Clues from these can raise the suspicion of the presence
of CTEPH and exclude other causes of dyspnea.
Diagnosis and Testing
If CTEPH is suspected, the next step is to perform ventilation–perfusion lung scintigraphy (V/Q
scan). This test has a high sensitivity of detecting perfusion defects and a high negative predictive
value, so CTEPH can be excluded if normal.
If the V/Q scan reveals perfusion defects, the next step would be to perform confirmatory
testing, which includes pulmonary computed tomography angiography (CTA) and right heart
catheterization with pulmonary angiography. Pulmonary CTA is effective in detecting thromboembolic material in the main and lobar segments of the pulmonary vasculature; however, it is less
sensitive in detecting segmental and subsegmental lesions.
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362 8—ACHD INTERVENTIONS
ial of medical
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Perfusion single photon emission computed tomography (SPECT)/CTA is a more recent
modality that integrates anatomic and perfusion data and provides additional data for diagnosis
and procedural planning
5
(Fig. 30.1). Right heart catheterization is needed to measure baseline
pulmonary pressures and pulmonary vascular resistance and directly image the pulmonary arteries
with pulmonary angiography. Pulmonary angiography is highly sensitive in detecting segmental
and subsegmental thromboembolic material, which is important for decision-making in treating
CTEPH.
How to Perform Pulmonary Angiography
1. Using a balloon wedge pressure catheter (Teleflex, Morrisville, NC) positioned in the right
or left pulmonary artery, insert a regular 0.0350 guidewire and exchange the balloon wedge
catheter for a 7F 145-cm pigtail.
2. Position the pigtail in the main left or right pulmonary artery. Connect the pigtail to a
power injector and inject 60 cc (610 cc) of contrast into each pulmonary artery.
3. In conventional biplane angiography, the left or right lung is placed in the isocenter with
straight left ascending oblique (LAO) and anteroposterior (AP) projections at a 90-degree
Presentation and testing (echocardiography,
PFT’s etc.) suspecting CETPH
CTA Pulmonary angiography
Central
thromboembolism
Surgical
thromboembolectomy
Fig. 30.1 Perfusion SPECT/CT angiography integrates anatomic and perfusion data and provides
additional data for diagnosis and procedural planning.
V/Q scan
high-probability PE
Central + peripheral
thromboembolism
Surgical thromboembolectomy
+/– PBA
Peripheral
thromboembolism
Tr
therapy
PBA
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angle. Image acquisition is set to the digital subtraction angiography mode at a rate of
four frames per second. The patient is asked to hold their breath, and contrast is injected.
Cine acquisition should be set up to have a long enough duration to acquire the pulmonary
flow in the arterial phase, followed by the levo-phase where pulmonary venous drainage
flow can be assessed.
4. As an alternative, rotational angiography can be performed using a C-arm that is equipped
with the capability and software to perform rotational image acquisition and image reconstruction. The right or left lung is again positioned in the isocenter. Contrast is injected
while cine acquisition is performed with the C-arm rotating around the isocenter, obtaining a series of x-ray images. With this modality, contrast is injected at 12 to 14 cc/second,
and image acquisition is initiated 2 seconds after the initiation of contrast delivery to adequately opacify the vasculature. The images are then reconstructed using special software
into a three-dimensional pulmonary tree. Rotational angiography permits a more thorough
assessment of overlapping vasculature and may assist in procedural planning and camera
positioning during subsequent interventions.
Pulmonary angiographic images are then reviewed to look for lesions that commonly present
as ostial narrowing, total occlusions, and hypoperfused arteries with poor microvascular blush and
delayed venous return.
Case Selection
Location and severity of lesions identified on CTA (for central lesions) and pulmonary angiography (for peripheral lesions) is key for the management of CTEPH. As a rule of thumb, the
more distal the involvement of thromboembolic material in the pulmonary tree, particularly in
segmental and subsegmental branches, the more challenging, higher-risk, and less successful
surgical thromboembolectomy becomes. Therefore thromboembolic material involving the central vasculature is a favorable anatomy for surgical thromboembolectomy, and this is the treatment
of choice. However, if the thromboembolic material involves the distal branches, then surgical
resection becomes very challenging and often not feasible.
Pulmonary balloon angioplasty is used in patients with thromboembolic lesions in the distal
pulmonary vessels that are not amenable for surgical resection. Angioplasty can also be offered in
a hybrid procedure for patients who have central and peripheral thromboembolic involvement,
whereby they undergo surgical resection of the central lesions and then are brought back for
balloon pulmonary angioplasty of residual disease in the segmental and subsegmental territories.
Medical therapy is the first step if the patient has distal involvement and is not found to be a
surgical candidate. Currently the only pharmacologic agent approved for CTEPH is riociguat, a
soluble guanylate cyclase stimulator. This promotes pulmonary artery dilatation. Patients are
given a trial of 6 to 8 weeks followed by a repeat right heart catheterization along with additional
objective reassessment of exercise limitation. If no improvement is observed with medical therapy,
pulmonary balloon angioplasty would be the next step.
Procedural Planning
Once the decision is made to proceed with pulmonary balloon angioplasty, careful procedural
planning is performed. This is a critical step and enough time should be spent planning, given
the complex three-dimensional anatomy of the pulmonary vasculature. Fundamental principles
should be applied in planning each procedure:
1. These procedures are to be divided into three to four sessions, given the risk of postangio-
plasty reperfusion pulmonary edema. Each session would target about three segmental
arteries and are to be spaced out by a minimum of 3 to 5 days, depending on the patient’s
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reserve, complexity of the anatomy, and results of previous interventions. Staggering inter-
ventions by longer periods of 2 or more weeks may provide an additional margin of safety.
2. These patients can be fairly ill at baseline and have low cardiopulmonary reserve; therefore
the initial balloon angioplasty procedural sessions should target vessels that are in the
distribution of the lowest perfusion territories on V/Q scan and can extract most benefit
from angioplasty, at the same time targeting lesions with favorable anatomy, allowing for
angioplasty to be done with the highest success rate and lowest complication rate. More
complex lesions can be tolerated better in later sessions as the patient’s hemodynamics
improve.
3. Cardiopulmonary reserve is determined by the severity of baseline mean pulmonary artery
pressure. Patients are less likely to tolerate even minor complications if they have higher
mean pulmonary artery pressure. Moreover, the higher the mean pulmonary artery pres-
sure, the higher the risk of procedural complications such as postrevascularization pulmo-
nary edema or bleeding from an inadvertent wire perforation. Therefore a conservative
approach is warranted for all patients, with closer attention to those with severely elevated
mean pulmonary artery pressures.
Step-by-Step Planning
1. Review the V/Q scan or the perfusion SPECT/CTA scan (if available: provides anatomic
and physiologic correlation). Identify territories with the lowest perfusion.
2. Review the pulmonary angiography and divide the pulmonary branches according to
conventional nomenclature as shown in Fig. 30.2.
3. Select target vessels on pulmonary angiography that correlate with areas of lowest perfusion
on V/Q scan or perfusion computed tomography (CT) scan.
4. Review the characteristics of each target vessel carefully with particular attention to
the lesion morphology and length. Classify these angiographic lesions using this novel
classification system suggested by Kawakami et al.
Type A: Ring stenosis
Type B: Webbed stenosis
Type C: Subtotal occlusion
Type D: Total occlusion
Type E: Tortuous, distal stenosis with cotton-wool stains of capillary arteries
The success rate and complication rate are associated with lesion type. Type A and B lesions
have up to 94% success rate with less than 3% risk of complication, whereas the success rate decreases and the complication rate increases with the other types of lesion morphologies, with up
to 52% rate of success and up to 15.5% risk of complications in types D and E.
This classification will help serve as a guide when choosing target lesions for initial sessions,
where the focus should be on selecting favorable anatomy associated with the highest success rate
and the lowest risk in the hopes of improving the pulmonary reserve of the patient so they tolerate angioplasty of more complex lesions at later sessions.
1. Lastly, before the procedure, baseline data should be recorded, including loudness of P2 on
physical examination; blood work, particularly NT proBNP, the level of which is directly
associated with the risk of postrevascularization pulmonary edema; and a 6-minute walk
test.
2. Warfarin is stopped 5 days before the procedure, and direct oral anticoagulants (DOACs)
are held 48 hours before the procedure and bridging with low-molecular-weight heparin
is performed.
3. Aspirin and clopidogrel are not required for the procedure. Previous anticoagulants are
reinitiated after the procedure, with bridging anticoagulation being used for warfarin.
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