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INNOVATION

Creation of a functional Potts shunt by stenting the

persistent arterial duct in newborns and infants
with suprasystemic pulmonary hypertension of
various etiologies
Heiner Latus, MD, Christian Apitz, MD, Axel Moysich, MD, Gunter Kerst, MD,
Christian Jux, MD, Juergen Bauer, MD, and Dietmar Schranz, MD

From the Pediatric Heart Center, Justus-Liebig University, Giessen, Germany.

KEYWORDS: BACKGROUND: The surgical creation of a Potts shunt has been reported in children with suprasystemic
pulmonary idiopathic pulmonary artery (PA) hypertension (IPAH) refractory to any medical therapy. This surgical
hypertension; approach allows acute decompression of the right ventricle (RV) and thereby avoids ventricular failure.
Potts shunt; We present 4 newborns and infants with different causes of pulmonary hypertension (PH) in whom
stenting the persistent interventional stenting of the patent ductus arteriosus (PDA) was used to create a “functional”
arterial duct Potts shunt.
METHODS: Suprasystemic PH was diagnosed by cardiac catheterization in 2 newborns with complex
left heart obstructive lesions, a patient with persistent PH of the newborn (PPHN), and an infant with
IPAH and was accompanied by RV dilation and imminent RV failure.
RESULTS: Stenting of the PDA was performed uneventfully during cardiac catheterization in all patients
and led to stabilization of clinical symptoms. The 2 patients with complex cardiac lesions subsequently
underwent successful biventricular repair. In the PPHN patient, the stented PDA was closed
interventionally when PA pressures dropped and a significant left-to-right shunt occurred. PA pressures
in the patient with IPAH remained high while the stented PDA still served as RV decompression in
avoidance of lung transplantation.
CONCLUSIONS: Newborns and infants with suprasystemic PH of varying etiologies may benefit from
the creation of a functional Potts shunt by stenting the PDA. This strategy should be considered in
patients with suprasystemic IPAH or PPHN as a temporary or permanent therapy when a PDA can be
identified at cardiac catheterization. It may also be beneficial in patients with PH due to left heart
obstructions by serving as a bridge to further corrective surgery.
J Heart Lung Transplant 2014;33:542–546
r 2014 International Society for Heart and Lung Transplantation. All rights reserved.

Creation of a Potts shunt has been propagated in children
with suprasystemic idiopathic pulmonary arterial (PA)
hypertension (IPAH) refractory to any medical therapy.1
Reprint requests: Dietmar Schranz, MD, Justus-Liebig-University of
Giessen, Pediatric Heart Center, Feulgenstr 12, D-35392 Giessen,
Germany. Telephone: -49-0-41-994-461.Fax: -49-0-641-994-3469.
E-mail address: dietmar.schranz@paediat.med.uni-giessen.de
1053-2498/$ - see front matter r 2014 International Society for Heart and Lung Transplantation. All rights reserved.
http://dx.doi.org/10.1016/j.healun.2014.01.860
Accordingly, this approach has primarily been indicated in
IPAH patients affected by right ventricular (RV) failure in
which this intervention was performed as a life-saving
procedure instead of atrial septostomy.2,3
Although data about long-term outcome are limited,3 the
Potts shunt principle has increased in popularity because
several theoretical advantages are provided compared with
atrial septostomy, including preserved oxygen supply for the
Latus et al. PDA Stunting in PAH
brain and the myocardium as well as a reduced risk of fatal
paradoxical embolic events. Another important benefit of
the Potts shunt may be the relief of RV pressure overload in
systole and, in part, also in diastole, with a subsequent
reduction in shifting of the interventricular septum to the left
ventricle (LV) with an improvement in systolic and diastolic
LV performance. Atrial septostomy, in turn, has no effect on
these unfavorable ventricular-to-ventricular interactions, and
the attempt to decompress the right atrium by atrial
septostomy remains ineffective, particularly in patients with
severe pulmonary hypertension (PH).
In newborns and infants, suprasystemic PA pressures can
be present in different conditions and also in association with
complex congenital cardiac lesions with the need for transient
or permanent relief of pulmonary pressure overload. Drawing
from recent advances in techniques4,5 and indications6 for RV
decompression by a Potts shunt, we describe our experience
with creation of a functional Potts shunt by stenting a
fortunately patent ductus arteriosus (PDA) in 4 patients with
suprasystemic PH of various etiologies.
Methods
Presented is a series of 4 patients with different causes of PH who
underwent cardiac catheterization at our institution between
November 2011 and May 2013.
Patient 1
In a 3-week-old newborn, suprasystemic PH was diagnosed in
conjunction with a small peri-membranous ventricular septal defect
(VSD) and mild mitral valve stenosis. Echocardiography showed
an enlarged and hypertrophied RV with impaired function. Across
the 2-degree tricuspid regurgitation, a systolic pressure gradient of
about 80 mm Hg was calculated by continuous-wave Doppler.
Across the slightly patent duct, a right-to-left shunt with a peak
Figure 1 Angiographic images show (Left) Patient 1 and (Right) Patient 2 after successful implantation of stents in the patent ductus
arteriosus (arrows). Note the right-to-left shunt through the stented patent ductus arteriosus to the descending aorta (*).
543
velocity of 2.5 m/sec was measured corresponding to a systolic
systemic artery pressure of 65 mm Hg. Medical treatment with
prostaglandin E, sildenafil, and oxygen was not effective.
Suprasystemic PA pressures (43/20 mm Hg vs 35/21 mm Hg
systemic pressure) were confirmed during cardiac catheterization,
and subsequently, the telescope technique was used to implant two
4-mm  15-mm Integrity stents (Medtronic, Minneapolis, MIN) in
the PDA. Transcutaneous oxygen saturation of the lower limb
decreased, from 91%-94% to 86%-89%, after the intervention. The
patient was discharged with sildenafil medication.
At the follow-up cardiac catheterization at the age of 8 months,
PA pressures had dropped to sub-systemic levels so that the stented
PDA was closed using a 5-4–mm Amplatzer Duct Occluder II
device (St. Jude Medical, Minneapolis, MN). Patch closure of the
VSD, including mitral valve reconstruction with papillary muscle
splitting, was achieved by corrective surgery at the age of 10
months.
All interventions were uneventful, and the patient recovered
quickly. Echocardiographic follow-up at the age of 13 months
showed almost normalized PA pressure (peak early diastolic
pulmonary regurgitation gradient of 25 mm Hg) in the presence of
preserved RV size and function.
Patient 2
Patient 2 was referred to our department at the age of 11 months
with diagnosed IPAH refractory to medical treatment with
sildenafil and bosentan and no further treatment option. Echocar-
diography revealed a pressure gradient of more than 130 mm Hg
across the tricuspid valve. A small atrial septal defect with right-to-
left shunt was visible. The hypertrophied RV showed still
preserved function. Cardiac catheterization revealed suprasystemic
PA pressures of 130/57/82 mm Hg and 106/57/78 mm Hg in the
ascending aorta and a small, 1-mm diameter PDA. After
preliminary balloon dilatation of the narrowed duct, a 5-mm
 12-mm Formula stent (Cook Medical, Bloomington, IN) was
implanted in the PDA, with reduction of the PA pressure to the
systemic level (Figure 1). Transcutaneous oxygen saturation was
544 The Journal of Heart and Lung Transplantation, Vol 33, No 5, May 2014
85% at the right arm (atrial shunt-dependent) and 75% at the
left leg.
The patient was subsequently treated with a PH-specific
combination therapy consisting of sildenafil, bosentan, and nifedi-
pine and was discharged home. Reevaluation at the age of 2.5 years
showed the patient was in a good clinical condition (New York
Heart Association functional class II), but still had a predominant
right-to-left shunt through the atrial septal defect caused by the
hypertrophied RV, with preserved systolic but impaired diastolic
function. Foot saturations were between 73% and 77%, and the
hemoglobin level had increased from 149 to 202 g/liter.
Echocardiography showed bidirectional flow through the PDA
stent, with a right-to-left shunt through the PDA during systole and
minimal left-to-right flow during diastole. Invasive pressure
measurements showed corresponding values, with pressures of
105/54/74 mm Hg in the PA and 92/53/67 mm Hg in the
descending aorta, respectively.
Patient 3
Patient 3 was diagnosed at the age of 1 week with Shone’s
complex, consisting of mitral stenosis and a borderline LV, a small
peri-membranous VSD, and a hypoplastic aortic arch with aortic
coarctation. On echocardiography, the enlarged RV showed
impaired systolic function. Cardiac catheterization revealed PA
and systemic pressure in the descending aorta of 62/30 mm Hg and
55/40 mm Hg, respectively, associated with a pulmonary capillary
wedge pressure of 20 mm Hg (left atrial pressure, 15 mm Hg; LV
end-diastolic pressure, 9 mm Hg). The small PDA was stented with
a 4-mm  18-mm Integrity stent (Figure 2). The patent foramen
ovale (PFO) was initially not balloon-dilated to avoid too much
left-to-right shunting (right atrial pressure, 4 mm Hg), thereby
providing enough pre-load for the small LV with the aim to support
its growth potential.
Oxygen saturation in the descending aorta immediately after
PDA stenting was 78%. At the follow-up catheterization 3 weeks
later, the LV diameter increased, PA pressure dropped to 44/20 mm
Hg, pulmonary capillary wedge pressure decreased to 8 mm Hg,
Figure 2 (Left) Angiographic image of the small patent ductus arteriosus (arrow) in Patient 3 and (Right) after successful implantation of
a 4-mm  18-mm Integrity (Medtronic, Minneapolis, MIN) stent (arrow) with a right-to-left shunt (*descending aorta).
and a left-to-right shunt through the VSD and the PDA was
observed in the presence of a systemic pressure of 55/28 mm Hg;
therefore, the PFO was dilated with a 7-mm Sterling balloon
(Boston Scientific, Natick, MA).
At the age of 10 months, the patient underwent biventricular
repair with aortic arch reconstruction. The arterial duct was
preoperatively closed by transcatheter PDA technique using a
4-4–mm Amplatzer Duct Occluder II. Recovery was uneventful,
and follow-up examinations showed preserved RV-function and a
left-to-right shunt through the still existing but restrictive VSD with
a pressure gradient of 60 mm Hg.
Patient 4
The fourth patient, who was diagnosed with persistent PH of the
newborn (PPHN), was referred to our center at the age of 2 weeks
under medical therapy with prostaglandin E to keep the PDA open.
Suprasystemic PA pressures were confirmed during catheterization
(107/47/67 mm Hg vs 79/43/57 mm Hg systemic pressure). A
4-mm  12-mm Integrity stent was implanted in the PDA without
any complications. Foot saturations were 91% to 92% before
intervention and 84% to 88% immediately after. Additional
PH-targeted medical therapy with sildenafil was started but
discontinued when 3 months later, PA pressures dropped to less
than half systemic level (47/18/27 mm Hg vs 110/50/78 mm Hg)
and echocardiography detected a large left-to-right shunt, which
led to incipient enlargement of left atrium and LV. Therefore, the
PDA-stent was closed interventionally using a 3-2–mm Amplatzer
Duct Occluder II. The PA pressure after closure decreased to
almost normal values of 33/12/20 mm Hg. The closure was favored
by an in-stent stenosis (Figure 3).
Discussion
Stenting of the persistent PDA is an established procedure in
the treatment of patients with a duct-dependent systemic or
pulmonary circulation.7–9 In this report, we successfully
Latus et al. PDA Stunting in PAH
Figure 3 (Left) Angiographic images of Patient 4 at the follow-up catheterization display a left-to-right shunt through the patent ductus
arteriosus stent (arrow) with an in-stent-stenosis and (Right) after successful closure using a 3-2–mm Amplatzer Duct Occluder II (arrow;
St. Jude Medical, Minneapolis, MN).
expanded the indication of PDA stenting by demonstrating
its usefulness in newborns and infants with different types of
severe PH. The implantation of a stent in the PDA served as
a functional Potts shunt and allowed for right-to-left
shunting, followed by unloading of the pulmonary circu-
lation, an increase in systemic output, and acute decom-
pression of the RV, thus avoiding ventricular failure.
Surgical creation of a Potts shunt has been propagated in
patients with severe and refractory IPAH to avoid or treat
RV failure.1–3 However, the implementation of such a shunt
requires open-chest heart surgery that carries a high peri-
procedural risk in these severely ill patients; therefore, new
approaches to solve this issue have recently been published.
Esch et al4 presented their experience with a novel catheter-
based technique for creating a connection between the left
PA and the descending aorta, which should reduce the risk
of a major surgical procedure. Boudjemline et al5 success-
fully performed PDA stenting in 3 patients with IPAH and
suprasystemic PA pressures in whom a still patent PDA was
found. In the series reported here, we were able to confirm
the usefulness and safety of PDA stenting in newborns and
infants with variant forms of PH.
With regards to the 11-month-old infant with IPAH in
our series and the 3 patients described by Boudjemline
et al,5 cardiac catheterization in IPAH patients should
include a careful lookout for a PDA, in particular in patients
with suprasystemic PA pressure. Regardless of the initial
option to use even a tiny PDA for duct stenting because of
the mentioned reasons, long-term patency of the PDA stent,
as well as its effect on outcome compared with surgical
procedures, needs to be determined in further studies that
include a larger number of patients.
Additionally, we were able to expand the principle of
Potts shunt using a transcatheter approach in 2 patients
with complex left heart obstructive lesions complicated by
545
suprasystemic PH as a bridge to further corrective surgery.
In a 2-week-old newborn with Shone’s complex, border-
line LV and a hypoplastic aortic arch, which was affected
by out-of-proportion postcapillary PH, PDA stenting was
performed to allow acute RV decompression in the
presence of impaired systolic RV and diastolic LV
function. This approach allowed functional recovery and
subsequent uncomplicated biventricular repair during
further follow-up. The same principle was used in a
3-week-old newborn with non-adaption of postnatal PH
caused by a post-capillary PH component due to mitral
stenosis.
The rationale behind these interventions was to decrease
postcapillary PH in borderline LV morphology by PDA
stenting, which led to decompression of the RV and a
subsequent improvement in LV diastolic function, followed
by an increase in cardiac output. Additional creation of a
communication between the right and the left atrium
allowed decompression of the LV by left-to-right shunting
through the defined atrial defect. The principle of this
procedure has already been published by our group in a
patient with borderline LV and suprasystemic PH.6
In another 2-week-old newborn, postnatal suprasystemic
PPHN was treated by PDA stenting as a temporary thera-
peutic strategy. RV failure could be avoided and diastolic
LV inflow improved. After biventricular unloading, RV and
LV function improved by beneficial interventricular inter-
action. It is important to note that prostaglandins E1 or E2
are frequently used in newborns with PPHN, particularly
because of their pulmonary vasorelaxant properties. The
additional ability of prostaglandins to maintain ductal
patency has also been described as beneficial in newborns
with PPHN and RV failure. However, in addition to the
adverse systemic hypotensive prostaglandin effects, it
usually does not allow for long-term treatment.
546 The Journal of Heart and Lung Transplantation, Vol 33, No 5, May 2014
The size of the PDA stent was chosen wide enough to
allow decompression of the RV but still guarantees
sufficient pulmonary blood flow and oxygenation. Our
experience with the hybrid approach in patients with
hypoplastic left heart syndrome7,9 leads us to hypothesize
that the size of the inverse Potts shunt has to be adapted to
the diameter of the descending aorta. Furthermore, the shunt
size is adapted according to the patient’s weight and the
residual function of the LV. In the described series, we
postulate a shunt size of 4 mm should be sufficient to reduce
suprasystemic pulmonary pressure immediately.
The potential advantages of the transcatheter PDA stent
are its less invasive approach compared with a surgical Potts
procedure, which carries a much lower risk for complica-
tions, and the possibility to close the stented PDA when PA
pressure decreases to a sub-systemic level. Closure of the
stented PDA by the transcatheter technique can easily be
performed after the right-to-left shunt across the duct is
changed to the preferential left-to-right shunt, which
demonstrates the apparent relief of out-of-proportion PH
by postponed physiologic post-natal adaptation. Remark-
ably, we observed in-stent stenosis in 1 patient before PDA
occlusion that facilitated the implantation of the duct
occluder and might be explained by the altered blood
oxygenation. Considering that the fetal circulation is
characterized by a relative hypoxemic environment and in
shunt-dependent circulation as, for example, in univentric-
ular hearts, the oxygen level is predominantly 75% to 85%;
thus, in-stent stenosis is rare in this context. However, the
fact that in-stent stenosis has been observed after right-to-
left shunt changed to left-to-right-shunt and, consequently,
that fully oxygenated blood passed the stented duct, suggest
that the higher oxygen saturation and higher oxygen partial
pressure resulted in a kind of physiologic endogenous
response to close the duct.
In conclusion, newborns and infants with suprasystemic
PH of varying etiologies may benefit from the creation of a
functional Potts shunt by stenting the PDA. This approach
allows acute decompression of the RV with unloading of the
pulmonary circulation and a subsequent increase in systemic
cardiac output. This strategy should be considered in
patients with suprasystemic IPAH or PPHN as a temporary
or permanent therapy when a PDA can be identified at
cardiac catheterization. It may also be beneficial in patients
with PH caused by obstructive left-sided heart disease by
serving as a bridge to further corrective surgery.
Disclosure statement
None of the authors has a financial relationship with a commercial
entity that has an interest in the subject of the presented manuscript
or other conflicts of interest to disclose.
References
1. Blanc J, Vouhe P, Bonnet D. Potts shunt in patients with pulmonary
hypertension. N Engl J Med 2004;350:623.
2. Labombarda F, Maragnes P, Dupont-Chauvet P, Serraf A. Potts
anastomosis for children with idiopathic pulmonary hypertension.
Pediatr Cardiol 2009;30:1143-5.
3. Baruteau AE, Serraf A, Levy M, et al. Potts shunt in children with
idiopathic pulmonary arterial hypertension: long-term results. Ann
Thorac Surg 2012;94:817-24.
4. Esch JJ, Shah PB, Cockrill BA, et al. Transcatheter Potts shunt creation
in patients with severe pulmonary arterial hypertension: initial clinical
experience. J Heart Lung Transplant 2013;32:381-7.
5. Boudjemline Y, Patel M, Malekzadeh-Milani S, Szezepanski I, Levy M,
Bonnet D. Patent ductus arteriosus stenting (transcatheter Potts shunt)
for palliation of suprasystemic pulmonary arterial hypertension: a case
series. Circ Cardiovasc Intervent 2013;6:e18-20.
6. Latus H, Apitz C, Schmidt D, et al. Potts shunt and atrial septostomy in
pulmonary hypertension caused by left ventricular disease. Ann Thorac
Surg 2013;96:317-9.
7. Michel-Behnke I, Akintuerk H, Marquardt I, et al. Stenting of the ductus
arteriosus and banding of the pulmonary arteries: basis for various
surgical strategies in newborns with multiple left heart obstructive
lesions. Heart 2003;89:645-50.
8. Schranz D, Michel-Behnke I, Heyer R, et al. Stent implantation of the
arterial duct in newborns with a truly duct-dependent pulmonary
circulation: a single-center experience with emphasis on aspects of the
interventional technique. J Intervent Cardiol 2010;23:581-8.
9. Akintuerk H, Michel-Behnke I, Valeske K, et al. Stenting of the arterial
duct and banding of the pulmonary arteries: basis for combined
Norwood stage I and II repair in hypoplastic left heart. Circulation
2002;105:1099-103.