Surgical-interventional hybrid orchestra consisting of Potts shunt, transcatheter tricuspid valve repair by Edwards-valve in a 26-year-old patient with pulmonary hypertension and right ventricular failure

doi:10.4236/wjcd.2013.36A001

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World Journal of Cardiovascular Diseases, 2013, 3, 1-4 WJCD

http://dx.doi.org/10.4236/wjcd.2013.36A001 Published Online September 2013 (http://www.scirp.org/journal/wjcd/)

Surgical-interventional hybrid orchestra consisting of

Potts shunt, transcatheter tricuspid valve repair by

Edwards-valve in a 26-year-old patient with

pulmonary hypertension and right

ventricular failure

Sabine Recla1, Blanka Steinbrenner1, Jennifer Schreier2, Stephan Fichtlscherer2, Dorle Schmidt1,

Christian Apitz1, Matthias Müller1, Juergen Bauer1, Hakan Akintuerk1, Dietmar Schranz1*

1Hessiches Kinderherzzentrum, Universitätskliniken Giessen, Frankfurt, Germany

2Medizinische Klinik III, Kardiologie Universitätsklinikum, Frankfurt, Germany

Email: *dietmar.schranz@paediat.med.uni-giessen.de

Received 30 June 2013; revised 5 August 2013; accepted 21 August 2013

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Reported here is a 26-year-old patient with corrected

congenital interrupted aortic arch admitted with

massive hemoptysis and severe suprasystemic pul-

monary hypertension provided him for heart-lung

transplantation. Initial closure of a small aorto-pul-

monary collateral remained ineffective. Persistent life-

threatening hemoptysis led to the decision for a modi-

fied Potts shunt with the aim of decompressing the

right ventricle and avoiding pulmonary-hypertensive

crisis. The hemoptysis did not recur. However, the

patient’s oxygen transport co ndition deteriorated and

necessitated an orchestra of interventional-surgical

approaches from re-coarctation stenting, Potts shunt

flow reducing to surgical and transcatheter tricuspid

valve repair with transient ECMO before the patient

could be discharged home.

Keywords: Pulmonary Hypertension; Potts Shunt;

Edwards Tricuspid Valve; Hybrid-Procedure

1. INTRODUCTION

Pulmonary hypertension (PH) with hemoptysis and

suprasystemic pressures is associated with poor progno-

sis [1-3]. Decrease of cardiac output is not only related to

impaired right ventricular (RV) function, but also a con-

sequence of impaired LV filling due to right-to-left ven-

tricular interaction [4]. As a novel approach for palli-

ating children with suprasystemic idiopathic pulmonary

arterial hypertension (PAH), surgical Potts shunt has

been reported as an alternative treatment to lung trans-

plantation [5,6].

2. CASE REPORT

A 26-year-old young man (body weight 52 kg) was em-

ergently referred with massive hemoptysis. He was

known to have suprasystemic pre-capillary PH secondary

to a complex congenital heart malformation (IAA-Type

B). Reconstruction of the aortic arch by a modified

Waldhausen technique combined with a pulmonary ar-

tery banding was performed in infancy. VSD closure and

pulmonary artery de-banding followed at the age of 2

years. 15 years later aortic arch was re-reconstructed by

PTFE-patch enlargement. He was known to have pre-

capillary pulmonary hypertension, which had been treated

by a calcium-antagonist, with the later addition of silde-

nafil and bosentan.

At admission life-threatening hemoptysis was asso-

ciated with right ventricular dilatation (143 ml/m2, MRI)

severe tricuspid valve insufficiency and significant cya-

nosis necessitating continuous supplemental oxygen

therapy. Heart catheterization was performed but only

one small collateral vessel could be detected and closed

by coils, but hemoptysis persisted. The suprasystemic

pulmonary arterial pressure (PAP) was confirmed with

PAP to systemic artery pressure (SAP) ratio of 1.2 and

1.5 after contrast medium injection which was used to

delineate the relationship between the left pulmonary

*Corresponding a uthor.

OPEN ACCESS

S. Recla et al. / World Journal of Cardiovascular Diseases 3 (2013) 1-4

artery (LPA) and descending aorta (Figure 1). Nitric

oxide test-inhalation (20 ppm) did not show any signifi-

cant effect on the PAH (PAP 102/63/76 mmHg; corre-

sponding PCWP of 16 mmHg, systemic arterial pressure

(SAP) in the ascending aorta of 95/61/73 mmHg, de-

scending aorta (DAO) of 84/58/66 mmHG). After de-

tailed discussion between the heart team, the patient and

his family led to the decision for a surgical Potts shunt

rather then listing the patient for combined lung- or heart-

lung transplantation. Based on the data from France [6],

a 13 mm PTFE-graft tube was surgically connected from

the left-PA to the DAO (Figure 2). The left-intercostal

surgical approach was technically uneventful. Hemopty-

sis was not observed again. However, the combination of

a post-operative left-lower lobe atelectasis, newly created

right-to-left shunt through the PTFE-tube and extreme

rarefied pulmonary vascular bed resulted in an extreme

lower body oxygen saturation of 28% distal to the Potts

shunt with an associated inferior caval vein (IVC) oxy-

gen saturation of 13%, but without an increased sys-

temic lactate level. The superior caval vein oxygen satu-

ration declined to 31% along with to a low left ventricular

cardiac output. The MRI blood-flow measurements in the

pulmonary artery demonstrated 61 ml/beat, 25 ml in the

ascending, and 12 ml in the descending aorta pre-, and

45 ml/beat post-Potts shunt, respectively. A reduced left

ventricular preload with consecutive low left ventricular

cardiac output was considered as a co-factor for the ex-

treme lower body desaturation. Therefore, the slightly re-

Figure 1. Demonstrates simultaneous injection of contrast me-

dium in the descending aorta (DAO) and left pulmonary artery

(LPA) to evaluate the morphology prior to Potts shunt surgery.

Measured LPA diameter was 24 mm, and DAO 16 mm, respec-

tively.

Figure 2. Depicts the surgical performed PTFE-graft after

connecting the LPA to the DAO.

coarctated descending aortic arch was stented by an An-

dra 39 mm XL stent mounted on a 20 × 45 mm Balloon-

in-Balloon (PFM). However, afterload reduction by di-

minishing the gradient across the aortic arch less than 5

mmHg improved only slightly the oxygen transport pa-

rameters. Therefore, we decided for flow reducetion

through the Potts shunt. A premounted 29 × 14 mm

Atrium graft stent (Advanta V12) advanced from a ve-

nous access was implanted in a diabolo shape by a slow

flared balloon inflation from out-side in until a narrowed

part of 7 mm in the middle of the graft-stent was

achieved. Thereafter measurements of oxygen transport

parameters improved. The additional trial to create an

interatrial communication for improving the left ven-

tricular preload by atrioseptostomy using the Brocken-

brough technique and gradual static balloon dilatation of

atrial septum resulted in ischemic ECG changes and

hemodynamic instability. Immediate device closure

(Amplatzer ASD-12 mm, AGA) was performed to treat

the hypoxemic reaction of the upper body part in par-

ticular of the coronary perfusion. The patient recovered

slowly but he remained bed-dependent as a NYHA func-

tional class of IV. Despite some clinical improvement the

tricuspid regurgitation increased progressively. Again,

surgical tricuspid valve reconstruction was considered as

an alternative to listing for tricuspid repair together with

lung transplantation. Surprisingly, the surgical approach

was well tolerated despite the need for an open-heart

surgery, However, the reconstructed tricuspid valve util-

izing a Carpentier-Edwards 30 mm band, remained suffi-

cient for just 2 - 3 weeks. Due to progressive grad III

regurgitation again, an off label use of a transcatheter 29

mm Edwards-Sapien Heart valve was considered to

S. Recla et al. / World Journal of Cardiovascular Diseases 3 (2013) 1-4 3

avoiding an additional open-heart surgery. Transcatheter

valve implantation was performed under general anes-

thesia with fluoroscopic and 3D-TEE guidance. An 18 F

Cook sheath was placed in femoral vein, and after sizing

of the Carpentier-band, a 39 mm Andra XXL stent

mounted on 30 × 60 mm balloon catheter (Balt) was po-

sitioned in the tricuspid-annulus and slight diabolo shape

created by expanding to 30 mm at both stent-ends fol-

lowed by using a 30 × 40 mm nucleus balloon (PFM).

The already prepared Edwards-valve was immediately

implanted within the previous Andra-stent and in the

metal band-marked tricuspid valve annulus. However,

the abrupt afterload increase after placement of the com-

petent Edwards-valve caused the right ventricular func-

tion to deteriorate. Balloon dilatation of the Atrium stent

within the Potts shunt to almost 9 mm diameter and In-

creasing dosages of catecholamines in addition to the

baseline inotropic therapy with milrinone and levo-

simendan failed to stabilize his circulation. Therefore,

the patient was put on ECMO (Extra-Corporal Mem-

brane Oxygenation) by utilizing the percutaneous femo-

ral vein (22 Fr sheath) and artery (16 Fr sheath) access.

Bi-ventricular and in particular right ventricular function

recovered over 5-days of ECMO therapy, and 7 days

ventilatory support. The “hybrid orchestra” of interven-

tional-surgical procedures is summarized on Figur e 3.

Three weeks later, after a hospitalization for a total of

4.5 months the patient was discharged home, but still

with a functional class NYHA III prior to further reha-

bilitation.

3. COMMENT

Patients with congenital heart disease dispose to PAH,

particularly if the cardiac defect is left unrepaired, but

even after corrective surgery due to inappropriate timing

or ineffective initial palliation [7]. Comparing patients

who could be transplanted and who died waiting had a

significantly higher incidence of supra-systemic right

heart pressures and hemoptysis [8,9], various rescue

treatments have been proposed. Atrial septostomy is cur-

rently recommended for bridging to lung transplantation

[10]. Recently, a Potts shunt has been described in pa-

tients with end-stage IPAH with suprasystemic PA pres-

sures who are refractory to medical treatment [5,6]. This

pulmonary-to-systemic connection changes cardiac path-

ophysiology into that of Eisenmenger’s physiology. At

our institution, Potts shunt combined with balloon atrial

septostomy was successfully performed in one other pa-

tient with out of proportion post-capillary PAH associ-

ated with a borderline LV-morpholo gy [11].

However, we underestimated the effect of the Potts

shunt on the oxygen transport parameters, and in par-

ticular the negative effect on the left ventricular preload.

Figure 3. Depicted are all utilized devices for several

transcatheter interventions. In neighborhood of the Andra-

stent placed in the aortic re-coarctation, the Jackson-coil in

a aortopulmonary collateral is shown; the ensemble of the

Andra- and Edwards-Sapien 29 mm valve positioned

within the Carpentier banded tricuspid valve slightly cov-

ert the 12 mm Amplatzer ASD-occluder (see text); addi-

tionally a 10 mm inflated high pressure balloon is shown

during slight re-dilatation of the Atrium V12-graft stent

which was previously placed within the Potts Shunt; the

dilatation of the graft-stent within the LPA-DAO-shunt to

10 mm was performed with the goal to reduce the afterload

of the failing right ventricle after sufficient Edwards-valve

placement.

Therefore, consecutive interventional-surgical measures

consisting of aortic coarctation stenting, Potts shunt flow

reduction; atrioseptostomy and immediate device closure,

surgical and finally transcatheter tricuspid valve repair

latest with the need for transient ECMO became neces-

sary. However, the right ventricle recovered with bal-

ancing of the right-left ventricular interacttion, which

allowed patients to discharge home.

This case highlights the utility o f a surgical-intervene-

tional hybrid strategy in the treatment of heart failure

with severe PAH, considered at the highest risk for ad-

verse surgical or even transplant outcomes. The short-

term efficacy of the appropriate trimmed Potts shunt and

the 29 mm-Edwards-valve in the tricuspid position in

severe PAH is reassuring.

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