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Right AP
Left AP
Fig. 30.2 Conventional nomenclature for pulmonary branches.
Right lateral
Left lateral
Procedural Steps
Figs. 30.3 to 30.10 present the steps necessary for this procedure.
1. The patient is prepped in the usual sterile fashion, and careful moderate sedation is initiated.
2. Access is obtained in the femoral vein using a 7F sheath placed using sterile technique. If
an inferior vena cava (IVC) filter is present, the procedure can still be carried out through
the filter. The right internal jugular vein is a potential option to use, but is technically more
challenging and exposes the proceduralist to higher levels of radiation.

366 8—ACHD INTERVENTIONS
Step 1:
target segments
Step 2:
angiograph
Step 3:
and flo
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3. Right heart catheterization is performed using a balloon wedge catheter. Pressures and
cardiac output using the Fick method are recorded.
4. The balloon wedge catheter is placed in the left or right pulmonary artery depending on
the site of intervention.
5. An exchange-length, extra-stiff wire is then inserted into the balloon wedge catheter.
6. The balloon wedge catheter is then exchanged over the stiff wire for an 8F shuttle sheath,
which is advanced with the dilator into the main left or right pulmonary artery.
7. The dilator and the exchange length wire are then removed and a 7F guiding catheter is
then inserted over a regular 0.0350 wire into the shuttle sheath with continuous flush.
Guide catheters of choice include a JR4 or JL4 for most of the interventions. In the
lower lobe branches, a multipurpose guiding catheter can occasionally help with better
engagement and reach.
8. Unfractionated heparin is given with a target activated clotting time (ACT) of 250 to
300 seconds.
9. The manifold is connected and pressure is monitored while the shuttle sheath and guid-
ing catheter are carefully manipulated to engage the target vessel branch. Pressure tracing
Fig. 30.3 Step-by-step approach to balloon angioplasty for chronic thromboembolic disease.
Planning
Selective
y
Lesion
w grade
Levo phase
Type A lesion
Pulmonary flow grade 1

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Step 4:
angioplasty
Step 5:
angiograph
Step 6:
of result
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Shuttle sheath
2.5 mm balloon
Balloon
JR 4 guide
catheter
Soft hydrophilic
wire
Selective
y
Evaluation
Fig. 30.4 Femoral venous access using a
7F sheath is obtained. Right heart catheter-
ization is performed using a balloon wedge
catheter. The balloon wedge catheter is then
placed in the left or right pulmonary artery,
depending on the site of intervention.
Levo phase
Pulmonary flow grade 3
No complications
Fig. 30.3 cont’d

368 8—ACHD INTERVENTIONS
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Fig. 30.5 An exchange-length, extra-stiff
wire is then inserted into the balloon
wedge catheter. The balloon wedge cath-
eter is then exchanged over the stiff wire for
an 8F shuttle sheath, which is advanced
with the dilator into the main left or right
pulmonary artery
Fig. 30.6 A guide catheter (usually JR4)
is then inserted over the stiff 0.350 wire
into the left or right pulmonary artery.
Fig. 30.7 Selective angiography is performed for the region of interest, with
careful documentation of antegrade
flow, lesion location and morphology,
and levo-phase, looking at venous drainage flow.

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Fig. 30.8 For pulmonary artery interventions, a hydrophilic soft-tipped wire with
a slightly larger than standard percutaneous coronary intervention tip (30 degrees and 4- to 5-mm primary curve) is
advanced across the stenotic lesions
under fluoroscopic vision.
Fig. 30.9 Once the distal wire position is
confirmed, balloon dilatation is performed,
starting from the distal to proximal vessel.
A 2.5 3 15 mm monorail compliant balloon is
used for the smaller distal vessel with multiple
brief low-pressure inflations.
Fig. 30.10 The 2.5-mm balloon is exchanged for a 4.0 3 15 mm monorail
compliant balloon to dilate the larger
proximal portion of the branches. These
two sizes suffice for a vast majority of interventions.

370 8—ACHD INTERVENTIONS
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is continuously checked to ensure no damping occurs before and after any injection. This
is a critical step in vessel injury complication avoidance, especially if the mean pulmonary
artery pressure is high. With the variation in the pulmonary artery tree anatomy, catheters
can abut the vessel wall, causing damping, and contrast injection into the thinner pulmonary vessel wall with high mean pulmonary artery pressure can be catastrophic.
10. Perform selective angiography, carefully documenting antegrade flow, lesion location and
morphology, and levo-phase, looking at venous drainage flow. A grading system has been
suggested to systematically classify pulmonary flow angiographically, called the pulmonary flow grade (Table 30.1).
7
Using this grading score, classify the flow according to the
pulmonary flow grading system. Lesion stenosis severity is most frequently determined
angiographically. Physiology of indeterminate-severity lesions can be assessed by a pressure wire placed across the lesion, just like the fractional flow reserve counterpart in
coronary artery disease assessment, with a cutoff value of less than 0.80 being a physiologically significant stenosis.
8
The pressure wire can be exchanged with the working wire
through a microcatheter or over-the-wire balloon.
11. For pulmonary artery interventions, a hydrophilic soft-tipped wire with a slightly larger
than standard percutaneous coronary intervention tip (30 degrees and 4- to 5-mm primary
curve) is advanced across the stenotic lesions under fluoroscopic vision. Tactile feedback is
often limited, given the size of the branches, and one should rely mostly on fluoroscopic
guidance, making sure that the wire does not enter overlapping small branches as it crosses
distally. If there is difficulty with support in crossing with the wire, a 2.5 3 15 mm compliant monorail angioplasty balloon can be advanced proximal to the wire tip to give more
support. Another way to increase support would be to carefully advance the shuttle sheath
and guiding catheter into a deeper position. If the lesion is still uncrossable with these
support strategies, particularly in severely fibrotic and complex lesions or subtotal/total
occlusions, wire escalation can be performed. Extra care should be taken when advancing
the stiffer wires, given that the risk of vessel injury increases. A typical learning curve of
30 to 40 procedures should be obtained before attempting complex lesions that require
significant wire escalation. Chronic total occlusions where the distal vasculature cannot be
visualized should likely not be attempted.
12. Once the distal wire position is confirmed, balloon dilatation is performed, starting from
the distal to proximal vessel. A 2.5 3 15 mm compliant monorail balloon is used for the
smaller distal vessel with multiple brief low-pressure inflations. The 2.5-mm balloon is
exchanged for a 4.0 3 15 mm monorail compliant balloon to dilate the larger proximal
portion of the branches. These two sizes suffice for a vast majority of interventions.
13. After that, repeat angiography is performed, looking at antegrade flow, adequacy of
modification of the stenotic sites, ruling out complications, and finally assessing for improvement in the venous return briskness. Postdilatation pulmonary flow grade is recorded.
14. In some situations, there may be significant recoil after balloon dilatation. Data for stent
placement are limited, and these are generally not required if there has been significant
improvement to flow even in the setting of tissue recoil.
TABLE 30.1 n Pulmonary Flow Grade
Grade 0 No arterial perfusion
Grade 1 Arterial perfusion present; however, no venous drainage
Grade 2 Arterial perfusion present with partial venous drainage
Grade 3 Arterial perfusion present with brisk venous drainage

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What Is an Acceptable Result?
1. Achievement of angiographic grade 3 flow in the target vessels.
2. If a pressure wire was used to assess lesion physiology, achievement of a postdilation pressure
gradient more than 0.8 is a marker of success.
3. No complications occurred, and less than 2 Gy of radiation and 300 mL of contrast were used.
Postoperative Care
The patient is admitted for overnight monitoring. The femoral vein sheath is removed after the
ACT drifts below 200. If the patient is on Coumadin, a dose is given that night and bridged with
low-molecular-weight heparin, with the first dose starting the next morning and continued until
the international normalized ratio (INR) is therapeutic again. If the patient is on a DOAC, it is
resumed the next morning to avoid vascular bleeding complications. A postprocedural chest x-ray
is obtained to screen for reperfusion pulmonary edema. Patients are usually dismissed the next day
unless they have another indication to stay within the hospital. A 6-minute walk test is performed
before the next session.
Complications
Pulmonary edema: This is a frequent complication of this procedure and is reported up to 60%
incidence in some studies.
abrupt exposure of the distal pulmonary vasculature to high pulmonary artery pressure after angioplasty, leading to reperfusion inflammatory injury that increases vessel permeability, causing
fluid extravasation across the capillaries. Risk factors for developing this complication include a
mean pulmonary artery pressure of more than 35 mmHg, elevated NT proBNP level, pulmonary
vascular resistance, and degree of change in the pulmonary flow grade after revascularization.
Pulmonary edema can vary in severity after angioplasty, and a grading system for reperfusion
pulmonary edema has been suggested by Inami et al. (Table 30.2).
Depending on the severity of the reperfusion pulmonary edema, patients are admitted to a
monitored inpatient bed or, if severe, the intensive care unit. Management of this complication is
challenging, and the choice of respiratory support depends on the severity of the pulmonary edema.
In extreme cases, there is cardiopulmonary collapse requiring extracorporeal membrane oxygenation
(ECMO). Although the mechanism of reperfusion pulmonary edema is inflammatory, diuretics can
be used to decongest the lungs and offload the right ventricle.
To lower the risk of developing this complication, the following methods can be used:
1. The higher the pulmonary pressure at baseline, the more conservative the strategy is for
angioplasty, as mentioned previously (more sessions, spaced out, and fewer target vessels in
each session).
9
From a pathophysiology standpoint, this is thought to be due to the
7
TABLE 30.2 n Reperfusion Pulmonary Edema Grade
Grade 1 No pulmonary edema on chest x-ray
Grade 2 Mild pulmonary edema on chest x-ray that improves with minimal oxygen for a few days
Grade 3 Moderate pulmonary edema on chest x-ray requiring high oxygen flows via a facemask to
Grade 4 Severe pulmonary edema on chest x-ray needing noninvasive positive-pressure ventilation
Grade 5 Extremely severe pulmonary edema requiring mechanical ventilation
maintain adequate oxygenation

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2. The administration of intravenous (IV) fluids is limited during the periprocedural
period, and diuretics are administered immediately if dyspnea occurs after successful
revascularization.
3. The use of the Pulmonary Edema Predictive Scoring Index (PEPSI) score as a predictor of
developing pulmonary edema has been suggested.
7
This score is calculated by multiplying the
sum total change in pulmonary flow grade by the baseline pulmonary vascular resistance. The
cutoff value for increased risk of reperfusion pulmonary edema was identified as 35.4. For
instance, if a patient had a pulmonary vascular resistance of 12 Wood units, to reach the cutoff
score of 35.4, using the PEPSI score formula, the calculated sum total change in pulmonary
flow grade would be 35.4 divided by 12, which leads to 2.95. This means that if the pulmonary
flow grade increases from 0 to 3 after angioplasty of the first target vessel, the risk of reperfusion pulmonary edema increases, and the procedure is ended. In other terms, the lower the
pulmonary vascular resistance (PVR), the more target vessel angioplasty can be performed.
4. Pressure wire has also been used in guiding the decision to stop angioplasty.7 If the post-
dilation pressure distal to the target lesion measured with a pressure wire reveals a mean
pressure of more than 35 mmHg, the risk of pulmonary edema increases.
The use of both the pressure wire method and the PEPSI score has been shown to be incremental in predicting the risk of pulmonary edema. Currently, there is no definitive evidence to
support this approach, and initial studies showed similar hemodynamic response and a decrease
in the number of sessions and treated lesions without reperfusion pulmonary edema compared
with the standard approach. These are not routinely used in most practices.
Vessel injury: Vessel injury comprises vessel perforation, rupture, or dissection. Perforation can
occur distally in the small vessels or proximally in the larger vessels from wire (either the 0.0140 wire
or stiff exchange wires) perforation, balloon oversizing, or, rarely, guiding catheter trauma. This
complication can be detected by contrast extravasation on pulmonary angiography. Any hemoptysis
should be taken seriously and can range from scant blood to overt hemoptysis. Depending on the
severity of the perforation, the first step is to secure the airways. If there is hemodynamic collapse,
ECMO is instituted. After that, the next step is to reverse anticoagulation and perform emergent
balloon tamponade proximal to or at the site of perforation. If the perforation is in a more proximal
position, covered stents can be deployed. If a perforation is in a small distal vessel, embolization by
coiling or other methods can be performed. In some instances, surgical intervention is required.
Other forms of vascular injury are rare and include guiding catheter– or wire-related dissection and
main pulmonary artery injury leading to cardiac tamponade. Rapid availability of pulmonary/
critical care and anesthesiology support for unanticipated complications is also important.
Outcomes of the Procedure
Although long-term data are still lacking, short-term data reveal that pulmonary balloon angioplasty is associated with improvement in hemodynamics and symptoms with an increase in the
6-minute walk distance.
10
Complications of this procedure depend on the type of lesion treated
and its morphology and range between less than 3% and up to about 15%.
and strategy, procedural mortality is infrequent. In our center, we had only one mortality at
30 days, which was not related to the procedure itself.
Summary and Take-Home Points
Pulmonary balloon angioplasty is a viable treatment strategy in patients with CTEPH with
distal thromboembolic lesions not amenable to surgical resection. Procedural planning is critical
and allows for performing this procedure safely and effectively.
n
Pulmonary balloon angioplasty is a therapeutic option for patients with CTEPH who have
a more peripheral (segmental and subsegmental) distribution of thromboembolic material.
6
With this technique

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n
Reperfusion pulmonary edema is a frequent complication that is associated with morbidity
and mortality. Careful planning is key to lowering the risk of this complication.
n
The higher the pulmonary artery pressure (.35 mmHg), the more conservative the
approach is, with more sessions and fewer target lesions per session.
n
V/Q scan and pulmonary angiography guide the target lesion selection, and initial sessions
should focus on simpler lesions that supply a bigger territory as determined by the perfusion
scan.
References
1. Saouti N, de Man F, Westerhof N, et al. Predictors of mortality in inoperable chronic thromboembolic
pulmonary hypertension. Respir Med. 2009;103(7):1013-1019.
2. Hoeper MM, Humbert M, Souza R, et al. A global view of pulmonary hypertension. Lancet Respir Med.
2016;4(4):306-322.
3. Ende-Verhaar YM, Cannegieter SC, Noordegraaf AV, et al. Incidence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: a contemporary view of the published literature.
Eur Respir J. 2017;49(2):1601792.
4. Lang IM, Dorfmuller P, Vonk Noordegraaf A. The pathobiology of chronic thromboembolic pulmonary
hypertension. Ann Am Thorac Soc. 2016;13 (Suppl 3):S215-221.
5. Yanagisawa R, Fetterly KA, Johnson GB, et al. Integrated use of perfusion SPECT/CTA Fusion imaging
and pulmonary balloon angioplasty for chronic pulmonary thromboembolism. JACC Cardiovasc Interv.
2017;10(5):532-534.
6. Kawakami T, Ogawa A, Miyaji K, et al. Novel angiographic classification of each vascular lesion in
chronic thromboembolic pulmonary hypertension based on selective angiogram and results of balloon
pulmonary angioplasty. Circ Cardiovasc Interv. 2016;9(10):e003318.
7. Inami T, Kataoka M, Shimura N, et al. Pulmonary edema predictive scoring index (PEPSI), a new index
to predict risk of reperfusion pulmonary edema and improvement of hemodynamics in percutaneous
transluminal pulmonary angioplasty. JACC Cardiovasc Interv. 2013;6(7):725-736.
8. Inami T, Kataoka M, Shimura N, et al. Pressure-wire-guided percutaneous transluminal pulmonary
angioplasty: a breakthrough in catheter-interventional therapy for chronic thromboembolic pulmonary
hypertension. JACC Cardiovasc Interv. 2014;7(11):1297-1306.
9. Kataoka M, Inami T, Hayashida K, et al. Percutaneous transluminal pulmonary angioplasty for the treatment
of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv. 2012;5(6):756-762.
10. Sugimura K, Fukumoto Y, Satoh K, et al. Percutaneous transluminal pulmonary angioplasty markedly
improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic
pulmonary hypertension. Circ J. 2012;76(2):485-488.

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Abstract: Chronic thromboembolic disease occurs when thromboemboli integrate into the wall
of the pulmonary arteries, resulting in obstruction in the pulmonary vasculature. CTEPH can
occur in the proximal, larger and/or distal, smaller pulmonary arteries. Surgical resection of the
occlusive lesions has been the standard of care of proximal CTEPH, while resection of distal
CTEPH is technically challenging. Pulmonary balloon angioplasty has emerged as an effective
option to treat distal CTEPH. It involves percutaneous techniques, using wires and coronary
artery balloon, to perform angioplasty of these distal lesions. Such procedures require careful
planning, including lesion selection and pulmonary edema preventative strategies. This chapter
reviews the steps in planning, execution and postoperative care in patients undergoing pulmonary
balloon angioplasty.
Keywords: Pulmonary Hypertension, CTEPH, Pulmonary balloon angioplasty
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