Progress in Pediatric Cardiology 33 (2012) 151–159

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Progress in Pediatric Cardiology

journal homepage: www.elsevier.com/locate/ppedcard

Pulmonary artery stents: Past, present and future

Matthew A. Crystal, Frank F. Ing ⁎
Texas Children's Hospital/Baylor College of Medicine, Houston, TX, United States

a r t i c l e i n f o a b s t r a c t

Keywords: Stents can be used to treat many forms of congenital heart disease, however, the majority of use remains in
Cardiac catheterization the pulmonary arterial system. Initial experimental work in the 1990's proved that stents were an effective
Pulmonary artery means for treating pulmonary artery stenosis. Subsequent experience demonstrated ways to overcome the
Stent limitations of small children, bifurcating stenoses, jailed side branches and the ways to cope with potential
Outcomes complications. We review the use of stents to rehabilitate the pulmonary arterial tree and outline the acute
and long-term results. Finally, we discuss the potential improvements and opportunities with bioabsorbable
stents and other future directions for bare metal stents as well.
© 2012 Elsevier Ireland Ltd. All rights reserved.

1. Background Premounted stents introduced in the mid-2000's could be passed

Mullins et al. first reported using stents to treat congenital heart
disease in 1991. Since then, the use of stents has expanded to include
not only pulmonary arteries and veins, but most systemic veins and
arteries as well. However, the majority of use remains in the
pulmonary arterial system in congenital heart disease.
In the early 1990's, only the Palmaz stent (J&J Medical, Warren,
NJ) was available along with balloons that typically required a 11 Fr
sheath. Patients were generally limited to 12 kg and larger. During
this period of the learning curve among pediatric interventionists,
some centers reported the technique of intraoperative stenting for
more difficult cases [1]. In the early 2000's, newer stents with
improved designs were introduced. The Genesis stent (J&J Medical,
Bloomington, MA) and the Doublestrut, MegaLD stents (EV3,
Plymouth, MN) have smoother edges, are more flexible and can be
delivered on smaller delivery systems. At the same time, balloons
with lower profiles and capable of high pressure dilations were also
introduced. These newer products permitted passage of the stent
through smaller delivery systems, thus lowering the size of patients
who can benefit from stents. New techniques of delivery were also
introduced to downsize the delivery system such as frontloading a
stent on a sheath and introducing that through a larger short sheath.
Another technique involved placing a dilator tip on the delivery
balloon and frontloading the stent on the delivery sheath [2]. The
dilator tip allowed a smooth transition for entrance of the entire
sheath, balloon and stent system through the skin as a single unit.
These newer stents, balloons and techniques permitted stents to be
delivered to the pulmonary artery of infants as small as 4.5 kg.
⁎ Corresponding author at: Baylor College of Medicine - Pediatric Cardiology Texas
Children's Hospital, 6621 Fannin Suite 19345-C, Houston, TX 77030, United States.
Tel.: + 1 832 826 5908.
E-mail address: fxing@bcm.edu (F.F. Ing).
through 6–7 Fr sheaths and were used in small infants as well.
However, these stents cannot be further dilated to the size of a
normal adult pulmonary artery limiting its use. Some have advocated
its use in patients who would require future cardiac surgery, such as
conduit replacements or Fontan completions [3,4].
In spite of low restenosis rates, stents placed into growing children
will require further dilation to keep up with normal somatic growth.
Intermediate and long-term follow up studies have shown excellent
results after further dilation over time. One long-term study
(15 year follow-up) showed the majority of patients remained
asymptomatic with RV pressures b1/2 systemic and having NYHA
class of I–II [5]. It is these excellent results that have lead to the
widespread use of stent implantation to the pulmonary arteries.
2. Anatomy/diagnosis
2.1. PA types
A thorough understanding of the heterogeneity of pulmonary
artery stenosis allows the interventionist to effectively plan the pul-
monary artery rehabilitation that is required. A combined strategy
of balloon angioplasty and stent implantation is required to effective-
ly address most lesions, but for the purposes of this review article we
will focus exclusively on the lesions amenable to stent implantation.
Other manuscripts have reviewed the four types of peripheral pulmo-
nary artery stenosis (type I — a single constriction of varying length
involving the main, right or left pulmonary artery; type II — lesions
at the pulmonary artery bifurcation of varying length; type III —
normal main and proximal pulmonary arteries with ostial stenosis
at multiple subsegmental branches; type IV — subsegmental stenoses
with concurrent central stenoses) [6,7]. In general, stents can be used
to treat stenoses at all levels except the subsegmental branches.

1058-9813/$ – see front matter © 2012 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ppedcard.2012.02.008
152 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159

2.2. Diversity of stenoses

Patients can present with native peripheral pulmonary artery

stenosis, post-operative stenosis or a combination of both. Under-
standing the different types of native peripheral pulmonary artery
stenosis is critical to selecting the appropriate balloon size, the type
and length of stent, and how best to manage implantation. Interroga-
tion of the diversity of post-operative changes (discrete stenosis ver-
sus long-segment hypoplasia, arterial folding/kinking, external
compression, stretching due to the LeCompte maneuver or intimal
flaps) is also critical in successfully managing pulmonary artery
stenosis, as each scenario can present its own inherent challenges to
overcome. Understanding the differences in compliance between a
fold with elastic properties and a discrete narrowing secondary to
calcification of an arterial patch or external compression by a dilated
neo-aorta can help avoid the pitfalls of stent malposition and balloon
rupture [6].

2.3. Genetic syndromes

There are additional challenges to face when consideration of ge-

netic syndromes is involved. Alagille syndrome, Williams syndrome
and other arteriopathies are particularly challenging due to the very
complex nature of the pulmonary stenoses (type III and IV) common-
ly associated with the condition [8,9]. The elastin anomalies associat-
ed with Williams syndrome cause multiple levels of stenosis that are
highly compliant and may be difficult to treat with traditional
angioplasty alone [9,10]. Many, but not all, children with Williams
syndrome will demonstrate improvement of peripheral pulmonary
stenosis over time, without requiring intervention. The patients
with Williams syndrome who require intervention are those with
near-systemic or suprasystemic right ventricular pressures in the
face of worsening hemodynamics and/or clinical symptoms related
to pulmonary obstruction [8]. Alagille syndrome has cardiovascular
defects noted in 85% of patients, of which complex peripheral pulmo-
Fig. 1. a: Diffuse bilateral branch pulmonary artery stenosis in a patient with Alagille
nary stenosis is most common. Unlike Williams syndrome, however,
syndrome. Stenoses can be seen at the main branch, lobar and segmental levels.
the pulmonary stenosis is unlikely to improve over time without Right ventricle pressures were suprasystemic prior to interventions. b: Same patient
intervention, and due to the associated liver disease, may be the who underwent bilateral stent implantation and further dilation over a two year
cause of death in up to 33% of patients. All children with Alagille period. Successful liver transplantation occurred after decrease in right ventricular
syndrome deserve cardiac assessment because of the high incidence pressures to less than half-systemic.

of congenital heart disease, of which, nonsurgical treatment is likely

to be more effective than surgical repair in this cohort of pulmonary
artery stenosis (Fig. 1). A catheter-based approach with angioplasty
and stent implantation, if appropriate, may improve long-term results
and improve liver transplant operative morbidity and mortality
[11,12].
2.4. Indications for stent implantation
In 2011, the American Heart Association published guidelines to
outline the indications for multiple interventional catheterization
procedures, including pulmonary artery stent implantation [13].
Determining who will benefit from intervention and when it is
indicated is the initial requirement. There is an important difference
between pulmonary artery rehabilitation in the patient with a biven-
tricular circulation as compared with the patient who underwent
single ventricle palliation. In this low flow state, pulmonary artery
gradients (measured indirectly by echocardiography or directly at
the time of catheterization) are unreliable markers for determining
severity. In the biventricular circulation, as well, a severe obstruction
of one branch pulmonary artery may be overlooked if it does not
demonstrate a significant gradient due to increased flow to the con-
tralateral unaffected lung.
A systematic approach, therefore, to treat pulmonary artery
stenosis will optimize results and minimize complications. Accepted
indications for pulmonary artery intervention in the biventricular
heart include a measurable gradient greater than 20 mmHg across
the region of stenosis, increased right ventricular pressure to approx-
imately two-thirds systemic and/or relative pulmonary blood flow
discrepancy between the two lungs with one lung having less than
35% of the total flow. Relative indications may also include pulmonary
valve insufficiency. In the patient who has undergone single ventricle
palliation, indications may include elevated proximal pressures
secondary to distal anatomic stenosis or relative pulmonary blood
flow discrepancy as described. Keeping in mind that there is not a
pumping chamber to push blood across the pulmonary arterial tree,
even mild gradients may be clinically significant. Additionally, special
circumstances such as persistent pleural effusions or chylothoraces,
low cardiac output state and protein-losing enteropathy may dictate
more aggressive management of subtle angiographic stenosis in the
absence of a pressure gradient [6,13].
Anatomic location plays a large role in determining the appropri-
ateness of balloon angioplasty alone compared with stent implanta-
tion. When stenosis involves the main pulmonary artery, attempts
with balloon angioplasty alone are typically performed as a first
step. This is to avoid impairing pulmonary valve function causing
free pulmonary insufficiency and to avoid jailing branch pulmonary
artery orifices. Under certain circumstances, such as bilateral branch
pulmonary artery stenosis, simultaneous bilateral stent implantation
can be very effective, but requires stent position within the main
pulmonary artery [14].
 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159
As well, in children with tetralogy of Fallot who are unfavorable
candidates for surgical intervention, a strategy of stenting the right
ventricular outflow tract is a feasible option [15]. If unsuccessful, or
if other reasons dictate the child requires cardiac surgical repair,
then these lesions are easily accessible to the surgeon and can be
addressed by patch angioplasty. Branch pulmonary arteries and distal
pulmonary architecture can initially be intervened upon with balloon
angioplasty alone, but if unsuccessful or if recurrence occurs, then
stent implantation can be a good long-term alternative (Fig. 2). A
thorough discussion with the cardiac surgeon in effectively planning
pulmonary artery rehabilitation is critical both before and after an ini-
tial surgical procedure. Being thoughtful, creative and well organized
in a collaborative environment will improve the likelihood of achiev-
ing long-term successful outcomes.
2.5. Choosing the appropriate stent and balloon
There are many different approaches that have been utilized and
documented in determining the most effective approach to stent
implantation and a thorough review of these techniques is beyond
the scope of this paper. As mentioned earlier, a systematic and
thorough approach, individualized to each patient, will allow for the
best result possible. Attention to detail is a very important key in
optimizing successful balloon inflation and stent implantation with
minimal complications. This refers to a thorough hemodynamic and
angiographic assessment at baseline. This is then followed by selec-
tion of an appropriate wire and long sheath to reach the target site.
We commonly employ an additional femoral vein site for a second
angiographic catheter for hemodynamic monitoring and angiography
Fig. 2. a. Long segment left pulmonary artery stenosis. b. Angioplasty with 6 mm balloon with no residual waist at end inflation. c. Angiogram post angioplasty demonstrating
vascular recoil with persistent stenosis. A stent is positioned in place for further support. d. left pulmonary artery with improved vessel caliber after stent implantation.
153
during and after stent implantation to avoid unnecessary catheter
and wire manipulations.
Selection of an appropriate size stent should always be based on
the eventual adult size vessel in which the stent is implanted. Selec-
tion of an open-cell stent versus a closed-cell stent is typically re-
served for the circumstance of jailing a side branch. The closed-cell
stents typically are easier to crimp onto a balloon and may have
slightly higher radial strength. On the other hand, the open-cell stents
allow dilation through its side cell into the orifice of the jailed side
branch [16] [17]. A thorough review of the different stent properties
is beyond the scope of this review, but an understanding of the differ-
ent properties can be helpful in dealing with the great variation found
in the pulmonary arterial tree. Occasionally, small patient size neces-
sitates smaller stents be placed with the understanding that further
surgical manipulation will be required. Most of the stents with a
large maximal diameter (dilatable to 18 mm) are unmounted, where-
as many of the small and medium stents (dilatable to 10–12 mm) are
premounted. These premounted stents, can go through smaller deliv-
ery systems (6–7 Fr), and have increased trackability and flexibility
that is advantageous in infants and small children [17–19]. Balloon
selection for the unmounted stents should include a length that
matches as closely as possible with the length of the stent to reduce
the likelihood of stent movement or balloon rupture. A non-
compliant balloon with higher rated burst pressure is better suited
to stent implantation, and a balloon-in-balloon catheter (Numed
Inc, Hopkinton, NY) can allow for a controlled deployment and
expansion of larger stents.
The catheter with the stent mounted on the balloon is advanced
through the long sheath, over the wire, to the target lesion. With
balloon inflation, the stent is expanded to the diameter of the balloon.
154 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159

With balloon deflation, the stent remains expanded within the

stenotic segment. Carefully removing the balloon with the assistance
of the sheath prevents stent malposition or embolization [20]. With
the sheath through the stent, simultaneous pressures can be obtained
through the end of the sheath and the angiographic catheter proximal
to the stent. Final angiography confirms the appropriate position of
the stent and assessment of the vascular lumen is performed. Main-
taining position of the long sheath distal to the stent allows for
post-implantation interventions as needed, limiting the likelihood of
stent movement. It should be emphasized that good wire position is
paramount even if it takes a few extra exchanges of catheters in
order to acquire an optimal wire position crossing the stenosis and
far into the subsegmental branches close to the diaphragm. Typically,
a stiff wire with a short floppy tip is used. In small children, such a
stiff wire may cause tricuspid and/or pulmonary valve regurgitation
and the wire will require pre-shaping or a softer wire to be used.
Some have advocated deployment of premounted stents without
the use of a long sheath [4]. While this technique may avoid hemody-
namic instability due to the stiffer long sheath, there is a higher risk of
stent malposition and embolization during balloon and wire removal.

3. Technical refinement

3.1. Simultaneous stent implantation

Early stenting for pulmonary arterial stenosis involved a single

Palmaz XD stent (J& J Med, Warren, NJ) mounted on a PEMT balloon
and delivered through a 11 Fr long sheath [21]. As experience
increased over time, this technology was applied to more complex
pulmonary arterial stenoses and in smaller children. In the case of
bilateral branch stenosis involving the orifices of the right and left
branches and whereby the branches are closely related, stenting one
side would jail the contralateral side and vice versa. Similarly, in
cases of stenoses involving two adjacent lobar branches, stenting
one lobar branch would jail the other. A technique of simultaneously
implanting two stents into both right and left branch pulmonary
arteries or into two adjacent lobar branches was developed by
Charles Mullins [14]. This technique involved the placement of two
delivery sheaths into the two adjacent branches and implanting
stents simultaneously in order to avoid jailing of either branch.
(Fig. 3) This technique also permits future access into both branches
for further dilation.

3.2. Jailing side branches

Occasionally, a stenosis spans across a side branch and angioplasty

is inadequate to relieve the obstruction. This is most commonly
encountered in long-segment proximal stenosis involving the right
or left branch whereby the stenosis to the lower lobes spans across
the orifice of the upper lobe branch. In that scenario, a stent would
offer structural support to improve flow to the lower lobes, but
would jail the orifice of the upper lobes. One strategy is to implant
Fig. 3. a. Bilateral branch pulmonary stenosis involving both orifices in a patient with
the stent to relieve the stenosis to the larger lower lobe segments
d-transposition following a Lecompte procedure. b. Bilateral simultaneous stent
but potentially compromising the flow to the smaller upper lobe seg- implantation of the proximal branch pulmonary arteries. c. Post bilateral stent implan-
ments. This is often a judgment call on the part of the interventionist tation angiogram demonstrating significant improvement in both orifice stenoses.
as a method of relieving pressure load on the right ventricle by
improving flow to the segments in the lower lobes. Intermediate
and long term follow up of these upper lobe branches indicate that
those with close to perpendicular angles to the main branch will The MegaLD and Doublestrut stents (EV3) have an “open-cell”
maintain normal flow while others with acute angles (b45°) resulted design that permits dilation of the side cells. In vitro experiments
in decreased flow or complete obstruction [5,22]. Overall, improve- indicate that these side cells can be dilated up to 12 mm in diameter.
ment of the right ventricular pressure following stent implantation These stents have proven to be particularly beneficial for side
was maintained, in spite of the decreased flow to the upper lobes. branches that are jailed by the side cells of a stent. As such, these
This is probably not dissimilar to the loss of the upper lobe following side cells can be easily dilated to improve flow to the jailed side
placement of a BT shunt in certain congenital heart diseases. branches (Fig. 4).
 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159 155

3.3. Use of cutting balloons

Occasionally, highly resistant stenotic lesions are encountered

whereby angioplasty is inadequate to “open” the stenotic segment.
In that case, implanting a stent may result in a residual stenosis with-
in the stent. One strategy to overcome these high resistant lesions is
to serially dilate first with cutting balloons (Boston Scientific, Natick,
MA) to “score” the stenotic segment, followed by dilation with high-
pressure balloons. Once the stenosis is relieved, then a stent can be
implanted. It is important to increase the size of the balloon in mm
increments after use with a cutting balloon to avoid over-dilation
and development of an uncontained dissection or aneurysm. Cutting
balloons are available in diameters up to 8 mm. Our experience
indicates that most high resistant lesions are found in unifocalized
aortopulmonary collaterals in PA/VSD and MAPCAs.

3.4. Techniques to minimize delivery system

Several techniques have been developed to minimize the stent

profile for delivery. This is particularly useful in small children and
infants. Larger and stiffer systems tend to prop open the tricuspid
and pulmonary valves and cause hypotension during its delivery.
Volume boluses and inotropes may be used to temporize the hemo-
dynamic compromise during stent delivery but can be quite precari-
ous and does not allow time to optimize stent position before
implant. Using umbilical tape to tighten the stent on a balloon allows
circumferential pressure to crimp the stent. Adding a small amount of
contrast onto the stent and waiting for it to dry also allows the stent
to “stick” onto the balloon so it does not shift during its advancement
inside the delivery sheath. Two additional techniques involve front-
loading the stent at the distal tip of the long sheath (Fig. 5). This tech-
nique avoids the need to advance the stent-balloon unit through the
entire length of the sheath where the stent can milk back on the bal-
loon. However, this technique requires a short sheath to be placed at
the groin that is 2 Fr sizes larger, in order to accommodate the long
sheath, and which still can be problematic in a small child or infant.
To overcome this issue, another technique involves front loading
the stent in the distal end of the long sheath and cutting off the dilator
tip and mounting that onto the balloon tip to act as a dilator during
insertion into the femoral vein (Figs. 6–7) [2,23]. This avoids the
need to use a larger short sheath at the groin. Since the introduction
of the Genesis XD stent, sheaths as small as 7 Fr can be used for
delivery using standard techniques.

3.5. Intraoperative stenting

Early in the experience, some reported the technique of intrao-

perative stenting. Currently, this technique falls into the category of
“hybrid procedures”. Current indications for intraoperative stenting
includes need for surgery on associated lesions such as a right ventri-
cle to pulmonary artery conduit replacement, Fontan completion, or
high risk lesions such as an early post-operative stenosis where the

Fig. 4. a. Angiogram demonstrating jailed left upper lobe with orifice stenosis after
stent implantation in proximal left pulmonary artery branch. An open-cell stent
(MaxLD) was implanted. b. Angioplasty of left upper lobe through the side cell of the
implanted stent. c. Angiogram of left upper lobe following angioplasty showing
improvement of the jailed orifice of the side branch.
Fig. 5. Front-loading technique: the stent-balloon unit is already positioned at the tip of
the long sheath and passed through the larger short sheath at the femoral access site.
The long sheath protects the stent-balloon unit as it is advanced over the guidewire
to the site of stenosis.
156 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159

Fig. 6. a. Dilator-tipped balloon front-loading technique: the dilator tip of the long
sheath is cut off and placed onto the balloon. The stent is mounted onto the balloon.
b. The stent-balloon unit is front loaded onto the sheath with the dilator tip acting as
the introducer dilator for the long sheath. c. This entire unit can be passed over a
guidewire from the femoral vein without requiring a larger short sheath.

risk of suture line rupture is high or in very small infants. Another

evolving indication includes treating the complex bifurcation steno-
ses involving the distal MPA and both ostia of the branch pulmonary
arteries in early infancy. As mentioned earlier, such lesions are com-
monly found in patients with Williams and Alagille syndrome. The
strategy is to place two stents into the branch pulmonary arteries si-
multaneously. The surgeon then trims the medial stent edges and
folds the lateral edges against the side of the main pulmonary artery.
This technique results in a single opening supported by the two mod-
ified stents from the main pulmonary artery into the two branches.
Not only does this technique permit adequate relief of the complex
stenosis, but also retains future access into both branches for further
dilation and rehabilitation of more distal stenoses commonly
associated with these two syndromes. Additionally, the likelihood of
balloon rupture from the contralateral stent is dramatically reduced,
if not eliminated.

4. Results

4.1. Acute results

Initial reports of successful pulmonary artery stent implantation

appeared in the literature in the early 1990's. This was followed
very quickly by acute outcomes and complications that were encoun-
tered with the initial patient cohort. The gradient across the stenosis Fig. 7. a. 4.5 month old (5.4 kg) infant post arterial switch operation with severe
decreased after stent implantation with a simultaneous increase in bilateral branch pulmonary artery stenosis. b and c. Large open-cell stent implanted
in both the proximal left pulmonary artery and right pulmonary artery using the
the vessel lumen. Results from Boston Children's Hospital and Texas
dilator-tipped balloon front-loading technique in this small infant. This technique
Children's Hospital showed the gradient was reduced from 55.2 ± allowed implantation of a large size stent into a small infant. Subsequent catheteriza-
33.3 to 14.2 ± 13.5 mmHg, and as much as 74 ± 24% at the Hospital tions will permit eventual dilation to 18 mm diameter. d. Post stent angiogram
for Sick Children. Simultaneous increase in the vessel lumen was demonstrating marked improvement in both branch pulmonary artery stenoses.
also greater than 100% in both studies [21,24]. This was a more
reliable outcome than the 58% success rate that had been reported
for balloon angioplasty alone and defined by Rothman et al. using
 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159
the criteria of a vessel lumen increase at least 50% of the pre-dilation
diameter, an increase of flow to the affected lung of at least 20% or a
decrease of the systolic right ventricular to aortic pressure ratio of
greater than 20% [25].
Reports also suggest that early post-operative pulmonary artery
stenoses can be treated effectively and safely using stents, especially
when compared with balloon dilation alone [26]. The patients who
underwent angioplasty alone were noted to have less success at
achieving a 50% increase in vessel diameter and had a high risk of
death due to vessel rupture. The stent implantation improved results,
while reducing mortality and risk in this high-risk patient group.
Stent implantation was also demonstrated to be effective in children
who had undergone single ventricle palliation. Interestingly, patients
who underwent stent implantation prior to a total cavopulmonary
connection and had the stent removed at surgery, required repeat
stent implantation at a subsequent catheterization. Therefore,
Kretschmar et al. recommended leaving previously implanted stents
in place or consideration of exchanging the stent for a larger stent
in a hybrid procedure. It is likely that the etiology of the initial steno-
sis may persist after surgery and that surgical removal of the stent
could result in vessel injury that promotes restenosis [27].
Additional work within major aortopulmonary collateral arteries
has demonstrated that pulmonary blood flow can be increased after
balloon angioplasty or stent implantation within stenotic collaterals,
but some vessels are more resistant to percutaneous techniques.
Overall, the procedure is safe.
5. Complications
The most common adverse events associated with pulmonary ar-
tery stenting are stent malposition and balloon rupture [21]. With
the initial Palmaz stents, balloon rupture was a significant concern
due to the relatively stiff stent and sharp edges. As stent and balloon
designs, have improved the risk of balloon rupture has decreased, but
is still an important factor in managing a successful stent implanta-
tion. In the case of balloon rupture, understanding how to stabilize
the balloon and reposition in a safe location for balloon exchanges
and eventually implantation is critical. Stent malposition is equally
as important, whether the stent milks off the balloon distally or
onto the shaft, at managing an effective implantation. The final step
of balloon removal can frequently be the time when a stent is malpo-
sitioned by either accidentally pulling the stent proximally out of
position during balloon or wire withdrawal or pushing the stent
further into the pulmonary artery when advancing a new larger
balloon into the stent for further dilation. Maintenance of good and
deep wire position allows stent repositioning, further dilation and
in some circumstances the implantation of additional stents or stent
removal [28].
At the initial implant procedure, or at subsequent redilation, vas-
cular tears/disruptions including dissection and aneurysm have
been reported [21,28]. Vascular injury is more often associated with
native branch pulmonary artery stenosis compared to post-
operative re-stenosis, especially when a long segment of hypoplasia,
typically resistant to dilation, is involved. Increased risk occurs with
high-pressure and over-sized balloons. Current recommendations
for stent implantation no more than 1–2 mm greater than the normal
adjacent segment or no more than 3–4× minimal stenotic diameter is
thought to reduce this risk due to the decreased stretch and tear of
the intimal layer. Successful balloon angioplasty has been demon-
strated by tears within the intimal layer of the artery. When this
layer is overly dilated or stented, increased intravascular pressure
can cause the formation of a pseudoaneurysm either acutely or
chronically. Additionally, stiff wires and the leading edge of the
balloon can also act as a potential cause of vascular disruption, and
care should be taken to avoid sudden forward “slips” which might
result in a distal small vessel disruption.
157
5.1. Long-term results/outcomes
Now that more than 20 years have passed since the first successful
pulmonary artery stent was implanted in humans, we are beginning
to have a better understanding of the longevity of stents in the
pulmonary position. The initial group of patients who received
pulmonary artery stents as part of the original Food and Drug
Administration (FDA) investigational device exemption (IDE) was re-
cently reported from Texas Children's hospital [5]. All patients with
data greater than 5 years were reviewed. Additionally, any patients
who died following the initial procedure were also included as an
intent-to-treat analysis. Overall, there were 43 surviving patients
13.2 ± 2.4 years after stent implantation and five who died following
their procedure. The average age at stent implant was 12.6 ± 7.1 years
with an average weight of 25 ± 13 kg. All patients received a Palmaz
“8-series” stent (J & J Interventional Systems, Warren, NJ). After the
initial catheterization, there was a significant improvement in pulmo-
nary artery size, gradient and percent stenosis (p b 0.001). Patients
underwent 1.4 ± 0.7 follow-up catheterizations and 1.2 ± 0.9 stent
re-dilations in order to accommodate somatic growth. The final result
demonstrated that the pulmonary artery size, gradient, percent ste-
nosis and the right ventricle to femoral artery pressure ratio remained
significantly improved compared to the baseline, pre-stent data.
Of the patients who did not survive, four of the deaths were due to
progression of their underlying cardiac condition and not associated
with their stents. One died as a result of a complication of redilation
(pulmonary artery fistula to aorta) during follow-up catheterization.
Surgical repair was unsuccessful. Additionally, there was one case of
dissection causing hemothorax, two pseudoaneurysms (one requiring
intervention, one small and stable not requiring intervention), balloon
rupture requiring balloon removal/retrieval and one patient had a
recurrence of an atrial arrhythmia. Of the survivors, 91% were in
New York Heart Association (NYHA) functional class I or II, 9% were
in class III and none were in class IV. There were 7 patients who under-
went surgical procedures (conduit revision, Fontan conversion) with
none requiring relief of pulmonary artery stenosis. Finally, jailed
vessels (at least partially) were noted in 49% of the stents with
one-third demonstrating diminished or absent flow, but still with a
significant improvement in right ventricular hypertension [5].
An additional concern has surrounded the growth of pulmonary
arteries after stent implantation and the ability to further dilate
implanted stents. It is well known that the fixed diameter of the
stented segments will not demonstrate growth, but a number of re-
ports have demonstrated the ability to redilate stents at subsequent
catheterizations in order to effectively match the intrinsic growth of
the proximal and distal segments as late as 10 years after implanta-
tion. McMahon et al. reported the risk of important neointimal prolif-
eration was 1.8% and restenosis was 2% [29]. It has been suggested by
this group, and confirmed by others, that a stent overdilated
compared to the adjacent normal vessel, minimal stent overlap
between adjacent stents and a sharp angle of the stent compared
with the vessel wall are all risk factors for neointimal hyperplasia
[24,30,31].
Recently, outcomes of fractured stents in the pulmonary arteries
have been reported. The incidence ranged from 2.5%, up to greater
than 20% for stents within the pulmonary arteries. McElhinney et al.
showed stent fracture to be relatively common and stent-related
risk factors included compression due to close proximity to the
ascending aorta, and a larger initial stent diameter [32,33]. He also
reported that 80% of patients with a stent fracture had a pressure
gradient; one-third of which were severe in nature. Breinholt et al.
demonstrated minimal stent fracture with only 71% being identified
by standard chest radiograph compared with dynamic fluoroscopy
(Breinholt JP 2008). Patients were asymptomatic without any
evidence for embolization of stent fragments. Further research will
be required to better understand this complex problem.
158 M.A. Crystal, F.F. Ing / Progress in Pediatric Cardiology 33 (2012) 151–159
6. Future/new developments
Stents have now been instrumental in improving the long-term
results of pulmonary artery rehabilitation, but it is well recognized
that stainless steel and platinum stents are not the final solution.
There are concerns of erosion, fracture, in-stent restenosis, and the
need for further dilation to match intrinsic growth in a child. With
this in mind, stents have been designed that will be able to optimize
the needed internal structure and support to effectively maintain an-
gioplasty effects, but dissolve over time without the need for surgical
removal. Biodegradable stents are not likely to replace the permanent
stents of the past 20 years, due to multiple factors previously outlined
including extrinsic compression or repetitive stress. Biodegradable
stents offer an excellent short-term solution and may obviate the
need for further dilation to account for patient growth. Currently,
there are no stents in the size required for branch pulmonary artery
stenoses. Furthermore, there is no data that shows the radial strength
of a biodegradable stent to be superior to current permanent stents
being used, but continued research and development is ongoing.
Although further innovation and adaptation will be required, the
development of biodegradable stents offers hope of a new era of
potential percutaneous interventions that were previously deemed
unsuitable [34,35].
In the interim, new strategies for overcoming the inherent limita-
tions of the vessels of small children have been developed by modify-
ing the implant techniques and the stents themselves. Previously,
implanted small stents that reached their maximal diameter in a
large vessel and became the limiting factor could only be treated sur-
gically. Maglione et al. have reported the use of the newer ultra-high-
pressure balloons (>20 atm pressures) to overcome the intrinsic
strength of maximally dilated stents and purposely fracture them.
This strategy allows the interventionist to successfully maintain the
stent in situ, but allow for further expansion with either balloon
angioplasty alone or additional stent reimplantation [36]. With
these higher risk maneuvers being employed, most labs around the
world have begun maintaining a stock of covered stents in case of
vascular dissection, rupture or pseudoaneurysm formation. The safety
of covered stents has been demonstrated in pulmonary arteries, and
has allowed creative use of standard stents, while minimizing risks
to children [19]. Unfortunately, balloon expandable covered stents
are still not available commercially in the USA. An additional strategy
has included the creation of an “open-ring” stent that is cut longitudi-
nally and then sewn back together with sutures that are absorbable
over a 60 day period (PDS II), thereby not ever developing a maximal
inflation diameter [37]. More recently, the “Growth stent” has ex-
panded on the open-ring design by manufacturing the two longitudi-
nal halves separately and then suturing them together with the same
PDS II sutures [38]. Use of this experimental stent in small children
has been reported although long-term data is still lacking [38].
7. Conclusion
Use of stents to treat branch pulmonary artery stenoses in congen-
ital heart disease has become a standard therapy since first reported
over two decades ago. During this time, newly designed stents and
balloons as well as innovative techniques of deployment have ex-
panded its use to infants and small children and in the most complex
of branch stenoses found in congenital heart disease. Initial and medi-
um term results from many institutions have proven to be excellent.
Although long-term data is limited to the past two decades, the
results to date show effective long-term relief of RV systolic hyper-
tension and maintenance of stent patency. Furthermore, new devel-
opments in biodegradable stents and covered stents will further
entrench the use of stents as the standard of care for treatment of
branch pulmonary artery stenosis. However, it is important to em-
phasize the need for early and close collaboration with the congenital
heart surgeons in order to map out the long-term treatment plan for
complex branch pulmonary artery stenoses.
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