http://www.jhltonline.

org

INNOVATION

Creation of a functional Potts shunt by stenting the

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 Accordingly, this approach has primarily been indicated in
with suprasystemic idiopathic pulmonary arterial (PA) IPAH patients affected by right ventricular (RV) failure in
hypertension (IPAH) refractory to any medical therapy.1 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
Reprint requests: Dietmar Schranz, MD, Justus-Liebig-University of
Giessen, Pediatric Heart Center, Feulgenstr 12, D-35392 Giessen,
Potts shunt principle has increased in popularity because
Germany. Telephone: -49-0-41-994-461.Fax: -49-0-641-994-3469. several theoretical advantages are provided compared with
E-mail address: [email protected] atrial septostomy, including preserved oxygen supply for the
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
Latus et al. PDA Stunting in PAH 543

brain and the myocardium as well as a reduced risk of fatal velocity of 2.5 m/sec was measured corresponding to a systolic
paradoxical embolic events. Another important benefit of systemic artery pressure of 65 mm Hg. Medical treatment with
the Potts shunt may be the relief of RV pressure overload in prostaglandin E, sildenafil, and oxygen was not effective.
systole and, in part, also in diastole, with a subsequent Suprasystemic PA pressures (43/20 mm Hg vs 35/21 mm Hg
reduction in shifting of the interventricular septum to the left systemic pressure) were confirmed during cardiac catheterization,
and subsequently, the telescope technique was used to implant two
ventricle (LV) with an improvement in systolic and diastolic
4-mm  15-mm Integrity stents (Medtronic, Minneapolis, MIN) in
LV performance. Atrial septostomy, in turn, has no effect on the PDA. Transcutaneous oxygen saturation of the lower limb
these unfavorable ventricular-to-ventricular interactions, and decreased, from 91%-94% to 86%-89%, after the intervention. The
the attempt to decompress the right atrium by atrial patient was discharged with sildenafil medication.
septostomy remains ineffective, particularly in patients with At the follow-up cardiac catheterization at the age of 8 months,
severe pulmonary hypertension (PH). PA pressures had dropped to sub-systemic levels so that the stented
In newborns and infants, suprasystemic PA pressures can PDA was closed using a 5-4–mm Amplatzer Duct Occluder II
be present in different conditions and also in association with device (St. Jude Medical, Minneapolis, MN). Patch closure of the
complex congenital cardiac lesions with the need for transient VSD, including mitral valve reconstruction with papillary muscle
or permanent relief of pulmonary pressure overload. Drawing splitting, was achieved by corrective surgery at the age of 10
from recent advances in techniques4,5 and indications6 for RV months.
All interventions were uneventful, and the patient recovered
decompression by a Potts shunt, we describe our experience
quickly. Echocardiographic follow-up at the age of 13 months
with creation of a functional Potts shunt by stenting a
showed almost normalized PA pressure (peak early diastolic
fortunately patent ductus arteriosus (PDA) in 4 patients with pulmonary regurgitation gradient of 25 mm Hg) in the presence of
suprasystemic PH of various etiologies. preserved RV size and function.

Methods Patient 2
Presented is a series of 4 patients with different causes of PH who
Patient 2 was referred to our department at the age of 11 months
underwent cardiac catheterization at our institution between
with diagnosed IPAH refractory to medical treatment with
November 2011 and May 2013.
sildenafil and bosentan and no further treatment option. Echocar-
diography revealed a pressure gradient of more than 130 mm Hg
Patient 1 across the tricuspid valve. A small atrial septal defect with right-to-
left shunt was visible. The hypertrophied RV showed still
In a 3-week-old newborn, suprasystemic PH was diagnosed in preserved function. Cardiac catheterization revealed suprasystemic
conjunction with a small peri-membranous ventricular septal defect PA pressures of 130/57/82 mm Hg and 106/57/78 mm Hg in the
(VSD) and mild mitral valve stenosis. Echocardiography showed ascending aorta and a small, 1-mm diameter PDA. After
an enlarged and hypertrophied RV with impaired function. Across preliminary balloon dilatation of the narrowed duct, a 5-mm
the 2-degree tricuspid regurgitation, a systolic pressure gradient of  12-mm Formula stent (Cook Medical, Bloomington, IN) was
about 80 mm Hg was calculated by continuous-wave Doppler. implanted in the PDA, with reduction of the PA pressure to the
Across the slightly patent duct, a right-to-left shunt with a peak systemic level (Figure 1). Transcutaneous oxygen saturation was

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 (*).
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 and a left-to-right shunt through the VSD and the PDA was
left leg. observed in the presence of a systemic pressure of 55/28 mm Hg;
The patient was subsequently treated with a PH-specific therefore, the PFO was dilated with a 7-mm Sterling balloon
combination therapy consisting of sildenafil, bosentan, and nifedi- (Boston Scientific, Natick, MA).
pine and was discharged home. Reevaluation at the age of 2.5 years At the age of 10 months, the patient underwent biventricular
showed the patient was in a good clinical condition (New York repair with aortic arch reconstruction. The arterial duct was
Heart Association functional class II), but still had a predominant preoperatively closed by transcatheter PDA technique using a
right-to-left shunt through the atrial septal defect caused by the 4-4–mm Amplatzer Duct Occluder II. Recovery was uneventful,
hypertrophied RV, with preserved systolic but impaired diastolic and follow-up examinations showed preserved RV-function and a
function. Foot saturations were between 73% and 77%, and the left-to-right shunt through the still existing but restrictive VSD with
hemoglobin level had increased from 149 to 202 g/liter. a pressure gradient of 60 mm Hg.
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
Patient 4
measurements showed corresponding values, with pressures of
105/54/74 mm Hg in the PA and 92/53/67 mm Hg in the The fourth patient, who was diagnosed with persistent PH of the
descending aorta, respectively. 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
Patient 3 (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
Patient 3 was diagnosed at the age of 1 week with Shone’s any complications. Foot saturations were 91% to 92% before
complex, consisting of mitral stenosis and a borderline LV, a small intervention and 84% to 88% immediately after. Additional
peri-membranous VSD, and a hypoplastic aortic arch with aortic PH-targeted medical therapy with sildenafil was started but
coarctation. On echocardiography, the enlarged RV showed discontinued when 3 months later, PA pressures dropped to less
impaired systolic function. Cardiac catheterization revealed PA than half systemic level (47/18/27 mm Hg vs 110/50/78 mm Hg)
and systemic pressure in the descending aorta of 62/30 mm Hg and and echocardiography detected a large left-to-right shunt, which
55/40 mm Hg, respectively, associated with a pulmonary capillary led to incipient enlargement of left atrium and LV. Therefore, the
wedge pressure of 20 mm Hg (left atrial pressure, 15 mm Hg; LV PDA-stent was closed interventionally using a 3-2–mm Amplatzer
end-diastolic pressure, 9 mm Hg). The small PDA was stented with Duct Occluder II. The PA pressure after closure decreased to
a 4-mm  18-mm Integrity stent (Figure 2). The patent foramen almost normal values of 33/12/20 mm Hg. The closure was favored
ovale (PFO) was initially not balloon-dilated to avoid too much by an in-stent stenosis (Figure 3).
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. Discussion
Oxygen saturation in the descending aorta immediately after
PDA stenting was 78%. At the follow-up catheterization 3 weeks Stenting of the persistent PDA is an established procedure in
later, the LV diameter increased, PA pressure dropped to 44/20 mm the treatment of patients with a duct-dependent systemic or
Hg, pulmonary capillary wedge pressure decreased to 8 mm Hg, pulmonary circulation.7–9 In this report, we successfully

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).
Latus et al. PDA Stunting in PAH 545

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 suprasystemic PH as a bridge to further corrective surgery.
its usefulness in newborns and infants with different types of In a 2-week-old newborn with Shone’s complex, border-
severe PH. The implantation of a stent in the PDA served as line LV and a hypoplastic aortic arch, which was affected
a functional Potts shunt and allowed for right-to-left by out-of-proportion postcapillary PH, PDA stenting was
shunting, followed by unloading of the pulmonary circu- performed to allow acute RV decompression in the
lation, an increase in systemic output, and acute decom- presence of impaired systolic RV and diastolic LV
pression of the RV, thus avoiding ventricular failure. function. This approach allowed functional recovery and
Surgical creation of a Potts shunt has been propagated in subsequent uncomplicated biventricular repair during
patients with severe and refractory IPAH to avoid or treat further follow-up. The same principle was used in a
RV failure.1–3 However, the implementation of such a shunt 3-week-old newborn with non-adaption of postnatal PH
requires open-chest heart surgery that carries a high peri- caused by a post-capillary PH component due to mitral
procedural risk in these severely ill patients; therefore, new stenosis.
approaches to solve this issue have recently been published. The rationale behind these interventions was to decrease
Esch et al4 presented their experience with a novel catheter- postcapillary PH in borderline LV morphology by PDA
based technique for creating a connection between the left stenting, which led to decompression of the RV and a
PA and the descending aorta, which should reduce the risk subsequent improvement in LV diastolic function, followed
of a major surgical procedure. Boudjemline et al5 success- by an increase in cardiac output. Additional creation of a
fully performed PDA stenting in 3 patients with IPAH and communication between the right and the left atrium
suprasystemic PA pressures in whom a still patent PDA was allowed decompression of the LV by left-to-right shunting
found. In the series reported here, we were able to confirm through the defined atrial defect. The principle of this
the usefulness and safety of PDA stenting in newborns and procedure has already been published by our group in a
infants with variant forms of PH. patient with borderline LV and suprasystemic PH.6
With regards to the 11-month-old infant with IPAH in In another 2-week-old newborn, postnatal suprasystemic
our series and the 3 patients described by Boudjemline PPHN was treated by PDA stenting as a temporary thera-
et al,5 cardiac catheterization in IPAH patients should peutic strategy. RV failure could be avoided and diastolic
include a careful lookout for a PDA, in particular in patients LV inflow improved. After biventricular unloading, RV and
with suprasystemic PA pressure. Regardless of the initial LV function improved by beneficial interventricular inter-
option to use even a tiny PDA for duct stenting because of action. It is important to note that prostaglandins E1 or E2
the mentioned reasons, long-term patency of the PDA stent, are frequently used in newborns with PPHN, particularly
as well as its effect on outcome compared with surgical because of their pulmonary vasorelaxant properties. The
procedures, needs to be determined in further studies that additional ability of prostaglandins to maintain ductal
include a larger number of patients. patency has also been described as beneficial in newborns
Additionally, we were able to expand the principle of with PPHN and RV failure. However, in addition to the
Potts shunt using a transcatheter approach in 2 patients adverse systemic hypotensive prostaglandin effects, it
with complex left heart obstructive lesions complicated by 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 cardiac output. This strategy should be considered in
allow decompression of the RV but still guarantees patients with suprasystemic IPAH or PPHN as a temporary
sufficient pulmonary blood flow and oxygenation. Our or permanent therapy when a PDA can be identified at
experience with the hybrid approach in patients with cardiac catheterization. It may also be beneficial in patients
hypoplastic left heart syndrome7,9 leads us to hypothesize with PH caused by obstructive left-sided heart disease by
that the size of the inverse Potts shunt has to be adapted to serving as a bridge to further corrective surgery.
the diameter of the descending aorta. Furthermore, the shunt
size is adapted according to the patient’s weight and the Disclosure statement
residual function of the LV. In the described series, we
postulate a shunt size of 4 mm should be sufficient to reduce None of the authors has a financial relationship with a commercial
suprasystemic pulmonary pressure immediately. entity that has an interest in the subject of the presented manuscript
The potential advantages of the transcatheter PDA stent or other conflicts of interest to disclose.
are its less invasive approach compared with a surgical Potts
procedure, which carries a much lower risk for complica- References
tions, and the possibility to close the stented PDA when PA
pressure decreases to a sub-systemic level. Closure of the 1. Blanc J, Vouhe P, Bonnet D. Potts shunt in patients with pulmonary
hypertension. N Engl J Med 2004;350:623.
stented PDA by the transcatheter technique can easily be
2. Labombarda F, Maragnes P, Dupont-Chauvet P, Serraf A. Potts
performed after the right-to-left shunt across the duct is anastomosis for children with idiopathic pulmonary hypertension.
changed to the preferential left-to-right shunt, which Pediatr Cardiol 2009;30:1143-5.
demonstrates the apparent relief of out-of-proportion PH 3. Baruteau AE, Serraf A, Levy M, et al. Potts shunt in children with
by postponed physiologic post-natal adaptation. Remark- idiopathic pulmonary arterial hypertension: long-term results. Ann
ably, we observed in-stent stenosis in 1 patient before PDA Thorac Surg 2012;94:817-24.
4. Esch JJ, Shah PB, Cockrill BA, et al. Transcatheter Potts shunt creation
occlusion that facilitated the implantation of the duct in patients with severe pulmonary arterial hypertension: initial clinical
occluder and might be explained by the altered blood experience. J Heart Lung Transplant 2013;32:381-7.
oxygenation. Considering that the fetal circulation is 5. Boudjemline Y, Patel M, Malekzadeh-Milani S, Szezepanski I, Levy M,
characterized by a relative hypoxemic environment and in Bonnet D. Patent ductus arteriosus stenting (transcatheter Potts shunt)
shunt-dependent circulation as, for example, in univentric- for palliation of suprasystemic pulmonary arterial hypertension: a case
series. Circ Cardiovasc Intervent 2013;6:e18-20.
ular hearts, the oxygen level is predominantly 75% to 85%; 6. Latus H, Apitz C, Schmidt D, et al. Potts shunt and atrial septostomy in
thus, in-stent stenosis is rare in this context. However, the pulmonary hypertension caused by left ventricular disease. Ann Thorac
fact that in-stent stenosis has been observed after right-to- Surg 2013;96:317-9.
left shunt changed to left-to-right-shunt and, consequently, 7. Michel-Behnke I, Akintuerk H, Marquardt I, et al. Stenting of the ductus
arteriosus and banding of the pulmonary arteries: basis for various
that fully oxygenated blood passed the stented duct, suggest
surgical strategies in newborns with multiple left heart obstructive
that the higher oxygen saturation and higher oxygen partial lesions. Heart 2003;89:645-50.
pressure resulted in a kind of physiologic endogenous 8. Schranz D, Michel-Behnke I, Heyer R, et al. Stent implantation of the
response to close the duct. arterial duct in newborns with a truly duct-dependent pulmonary
In conclusion, newborns and infants with suprasystemic circulation: a single-center experience with emphasis on aspects of the
PH of varying etiologies may benefit from the creation of a interventional technique. J Intervent Cardiol 2010;23:581-8.
9. Akintuerk H, Michel-Behnke I, Valeske K, et al. Stenting of the arterial
functional Potts shunt by stenting the PDA. This approach duct and banding of the pulmonary arteries: basis for combined
allows acute decompression of the RV with unloading of the Norwood stage I and II repair in hypoplastic left heart. Circulation
pulmonary circulation and a subsequent increase in systemic 2002;105:1099-103.